ENSMUSG00000027204


Mus musculus

Features
Gene ID: ENSMUSG00000027204
  
Biological name :Fbn1
  
Synonyms : Fbn1 / Fibrillin-1 Asprosin / Q61554
  
Possible biological names infered from orthology : fibrillin 1 / P35555
  
Species: Mus musculus
  
Chr. number: 2
Strand: -1
Band: F1
Gene start: 125300594
Gene end: 125507993
  
Corresponding Affymetrix probe sets: 10487040 (MoGene1.0st)   1425896_a_at (Mouse Genome 430 2.0 Array)   1438870_at (Mouse Genome 430 2.0 Array)   1458593_at (Mouse Genome 430 2.0 Array)   1460208_at (Mouse Genome 430 2.0 Array)   
  
Cross references: Ensembl peptide - ENSMUSP00000028633
Ensembl peptide - ENSMUSP00000099524
NCBI entrez gene - 14118     See in Manteia.
MGI - MGI:95489
RefSeq - XM_011239290
RefSeq - NM_007993
RefSeq - XM_006498747
RefSeq Peptide - NP_032019
swissprot - Q61554
Ensembl - ENSMUSG00000027204
  
See expression report in BioGPS
See gene description in Wikigenes
See co-cited genes in PubMed


Ortholog prediction (from Ensembl)
Ortholog nameID Species
 fbn2aENSDARG00000051896Danio rerio
 FBN1ENSGALG00000004960Gallus gallus
 FBN1ENSG00000166147Homo sapiens


Paralog prediction (from Ensembl)
Paralog nameIDSimilarity(%)
Fbn2 / Q61555 / Fibrillin-2 Fibrillin-2 C-terminal peptide / P35556* / fibrillin 2*ENSMUSG0000002459869
Ltbp2 / latent transforming growth factor beta binding protein 2 / Q14767*ENSMUSG0000000202017
Ltbp4 / Q8K4G1 / Latent-transforming growth factor beta-binding protein 4 / Q8N2S1*ENSMUSG0000004048816
Ltbp1 / Q8CG19 / Latent-transforming growth factor beta-binding protein 1 / Q14766*ENSMUSG0000000187016
Ltbp3 / latent transforming growth factor beta binding protein 3 / Q9NS15*ENSMUSG0000002494012
Fbln2 / P37889 / Fibulin-2 / P98095*ENSMUSG0000006408011
Fbln1 / Q08879 / Mus musculus fibulin 1 (Fbln1), transcript variant 2, mRNA. / P23142* / fibulin 1*ENSMUSG000000063697
Efemp1 / Q8BPB5 / EGF-containing fibulin-like extracellular matrix protein 1 / Q12805* / EGF containing fibulin extracellular matrix protein 1*ENSMUSG000000204674
Efemp2 / Q9WVJ9 / EGF-containing fibulin-like extracellular matrix protein 2 / O95967* / EGF containing fibulin extracellular matrix protein 2*ENSMUSG000000249094
Fbln5 / Q9WVH9 / Fibulin-5 / Q9UBX5*ENSMUSG000000211864


Protein motifs (from Interpro)
Interpro ID Name
 IPR000152  EGF-type aspartate/asparagine hydroxylation site
 IPR000742  EGF-like domain
 IPR001881  EGF-like calcium-binding domain
 IPR009030  Growth factor receptor cysteine-rich domain superfamily
 IPR013032  EGF-like, conserved site
 IPR017878  TB domain
 IPR018097  EGF-like calcium-binding, conserved site
 IPR026823  Complement Clr-like EGF domain
 IPR036773  TGF-beta binding (TB) domain superfamily


Gene Ontology (GO)
TypeGO IDTermEv.Code
 biological_processGO:0001501 skeletal system development IEA
 biological_processGO:0001656 metanephros development IEA
 biological_processGO:0001822 kidney development IEA
 biological_processGO:0007507 heart development IEA
 biological_processGO:0009653 anatomical structure morphogenesis IBA
 biological_processGO:0010469 regulation of signaling receptor activity IEA
 biological_processGO:0033627 cell adhesion mediated by integrin IEA
 biological_processGO:0035582 sequestering of BMP in extracellular matrix IMP
 biological_processGO:0035583 sequestering of TGFbeta in extracellular matrix IMP
 biological_processGO:0043010 camera-type eye development IEA
 biological_processGO:0045671 negative regulation of osteoclast differentiation IEA
 biological_processGO:0048048 embryonic eye morphogenesis IEA
 biological_processGO:0048050 post-embryonic eye morphogenesis IEA
 biological_processGO:0071560 cellular response to transforming growth factor beta stimulus IEA
 biological_processGO:1990314 cellular response to insulin-like growth factor stimulus IEA
 biological_processGO:2001205 negative regulation of osteoclast development IEA
 cellular_componentGO:0001527 microfibril IEA
 cellular_componentGO:0005576 extracellular region ISO
 cellular_componentGO:0005604 basement membrane IEA
 cellular_componentGO:0005615 extracellular space IEA
 cellular_componentGO:0031012 extracellular matrix IEA
 molecular_functionGO:0005178 integrin binding IEA
 molecular_functionGO:0005179 hormone activity IEA
 molecular_functionGO:0005201 extracellular matrix structural constituent IBA
 molecular_functionGO:0005509 calcium ion binding ISO
 molecular_functionGO:0005515 protein binding IPI
 molecular_functionGO:0008201 heparin binding IEA
 molecular_functionGO:0042802 identical protein binding IEA
 molecular_functionGO:0044877 protein-containing complex binding IEA


Pathways (from Reactome)
Pathway description
Degradation of the extracellular matrix
Elastic fibre formation
Molecules associated with elastic fibres
Integrin cell surface interactions
Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
Post-translational protein phosphorylation


Phenotype (from MGI, Zfin or HPO)
IDPhenotypeDefinition Genetic BG
 MP:0000063 reduced bone density "decreased mineral content of bone; indicator of bone strength" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, MGI:CLS, J:57315]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0000130 abnormal cancellous bone morphology "structural anomaly of bone that has a latticelike or spongy structure" [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1tm1Itl/Fbn1tm1Itl
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0000150 abnormal rib morphology "malformed bones forming the bony wall of the chest" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8, J:50311]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0000160 kyphosis "forward curvature of the spine, characterized by extensive flexion. " [J:62023, J:66943, Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8, cml:Cathleen M. Lutz , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0000163 abnormal cartilage morphology "anomalous structure or development of the nonvascular, resilient, flexible connective tissue found primarily in joints, walls of the throax, and tubular structures, but which also comprises most of the skeleton in early fetal life " [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0000266 abnormal cardiac morphology "anomalies in the hollow, muscular organ that maintains the circulation of the blood" [MeSH:National Library of Medicine - Medical Subject Headings , 2003]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0000272 abnormal aorta morphology "structural anomaly of the main trunk of the systemic arteries" [MeSH:National Library of Medicine - Medical Subject Headings , 2003, cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0000274 enlarged heart "increase over normal size of the heart" [J:29971]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0000276 right ventricle hypertrophy "increased size of the right ventricle" [J:33629]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0000286 abnormal mitral valve morphology "malformation of the valve between the left atrium and the left ventricle of the heart" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:53370]
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Allelic Composition: Fbn1tm1Hcd/Fbn1tm1Hcd
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0000324 increased mast cell number "greater than expected number of the granulated cells found in most tissues and which contain large amounts of histamine and heparin in granules" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0000371 diluted coat color "a coat color that appears lighter in intensity or paler than normal" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+,KitW/Kit+
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0000439 enlarged skull 
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0000538 abnormal urinary bladder morphology "malformation of the inflatable musculomembranous bag for holding urine" [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0001175 abnormal lung morphology "malformed organ of respiration" [J:35802]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0001176 abnormal lung development "malformation or arrest of differentiation of the organs of respiration" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:54931]
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Allelic Composition: Ins1tm1Jja/Ins1tm1Jja,Tg(Ins2*Y16A)1Ell/0
Genetic Background: NOD.Cg-Ins1tm1Jja Tg(Ins2*Y16A)1Ell

 MP:0001179 thicker alveolar septa 
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0001182 lung hemorrhage "bleeding in the respiratory organs" [J:66345]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0001183 overexpanded alveoli "expanded volume of the saclike terminal dilation of the respiratory bronchioles" [J:66345]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1Tsk/Fbn1+,Il4ratm1Sz/Il4ratm1Sz
Genetic Background: involves: BALB/cJ * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Il4ratm1Sz/Il4ra+
Genetic Background: involves: BALB/cJ * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Tgfb1tm1N/Tgfb1+
Genetic Background: involves: 129S/SvEv * C57BL/6J * C57BL/10 * DBA/2

 MP:0001191 abnormal skin condition "anomalous state of the skin" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0001198 tight skin "skin has a taut, stretched appearance" [J:65038]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/Kit+
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0001200 thick skin "greater depth of skin " [J:47225]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,Il4ratm1Sz/Il4ra+
Genetic Background: involves: BALB/cJ * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

 MP:0001222 epidermal hyperplasia "increase in size of the epidermis due to increase in epidermal cell number" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:65146]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0001243 abnormal dermal layer morphology "malformed or atypical condition of the dermal layer of the skin" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:56777]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/JKcc

Allelic Composition: Fbn1Tsk/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * C57BL/6J

Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0001245 thick dermal layer "increased depth of the dermis" [J:65033]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0001267 enlarged chest "wider diameter, or distended chest cavity" [J:18048]
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Allelic Composition: Sufutm1Rto/Sufutm1Rto
Genetic Background: involves: 129X1/SvJ * C57BL/6

 MP:0001505 hunched posture "stooped low with the limbs pulled in close to the body and arched back" [J:67231, J:30404]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0001577 anemia "less than normal levels of red blood cells, hemoglobin or volume of packed red blood cells " [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:53370]
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Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0001613 abnormal vasodilation "anomalous widening of the lumen of the blood vessels" [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: F11tm1Gjb/F11tm1Gjb
Genetic Background: B6.129X1-F11tm1Gjb

 MP:0001614 abnormal vasculature "anomalies in the structure of development of the network of tubes that carries blood through the body" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1Hcd/Fbn1tm1Hcd
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0001625 cardiac hypertrophy "an increase in size of the cardiac tissue, not due to increased cell number " [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0001762 polyuria "increased volume of urine produced and excreted" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator, smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0001861 lung inflammation "local accumulation of fluid, plasma proteins, and leukocytes in the lung" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator, Immunobiology , The Immune System in Health and Disease:ISBN 0-8153-1691-7]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0001914 hemorrhage "loss of blood from the vascular compartment to the exterior or into nonvascular body space as a result of rupture or severance of the blood vessels" [Stedman s Medical Dictionary, 27th edition:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0001943 abnormal respiration "anomaly in the movement of gases into and out of the lung " [Stedman s Medical Dictionary:ISBN 0-683-40008-8, cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn2tm1Rmz/Fbn2tm1Rmz
Genetic Background: either: (involves: 129/Sv) or (involves: 129/Sv * C57BL/6J)

 MP:0001954 respiratory distress "physical difficulty or inability to breathe" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:54931]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0001958 emphysema "lung disease characterized by increased size of terminal bronchioles with destructive changes in their walls and reduction in number" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:33629]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * C57BL/6 * CD-1

Allelic Composition: Fbn1Tsk/Fbn1+,Stat6tm1Gru/Stat6tm1Gru
Genetic Background: involves: 129S2/SvPas * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Il4tm1Nnt/Il4tm1Nnt
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Tg(TcraH-Y,TcrbH-Y)71Vbo/?
Genetic Background: involves: C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Tg(Tcra2C,Tcrb2C)1Dlo/?
Genetic Background: involves: C57BL/10 * DBA/2

 MP:0002060 abnormal skin morphology "malformation or atypical structure of the membranous protective covering of the body" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8, J:56777]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

 MP:0002080 prenatal lethality "death anytime between fertilization and E18.5" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Tnfsf11tm1Ywc/Tnfsf11+
Genetic Background: involves: 129P2/OlaHsd

 MP:0002083 premature death "death after 3 weeks of age, but before normal life span" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Sufutm1Rto/Sufutm1Rto
Genetic Background: involves: 129X1/SvJ * C57BL/6

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0002127 abnormal cardiovascular system morphology "abnormal development of the heart or vascular tissue resulting in morphological abnormality" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Ubl4atm1.1Xya/Y
Genetic Background: involves: 129 * C57BL/6 * FVB/N

 MP:0002267 abnormal bronchiole morphology "any structural anomaly of the conducting airway of the lungs found terminal to the bronchi. These structures contain neither cartilage nor mucous-secreting glands; the epithelium of the bronchioles becomes thinner with each branching. " [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator, Pathology , 2nd edition:ISBN 0-397-51047-0]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0002270 abnormal alveoli morphology "any structural anomaly of the outpouching of the respiratory system where gas exchange occurs" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0002279 abnormal diaphragm morphology "structural defect in the thin musculomebraneous barrier that separates the abdominal and thoracic cavities; these muscles often used for breathing control" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8, cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0002280 abnormal intercostal muscle morphology "anomaly in the respiratory muscles that arise from the lower border of one rib and insert into the upper border of the adjoining rib " [MeSH:National Library of Medicine - Medical Subject Headings , 2003, csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0002398 abnormal bone marrow cell morphology/development "anomalous structure or formation of the cells found in the bone marrow" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm1Itl/Fbn1tm1Itl
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0002433 abnormal T helper 1 morphology/development "anomalous structure, formation, or numbers of the subset of helper T cells that enhance cell-mediated immune responses. These cells secrete IL-1 and gamma-IFN and are generally involved in inflammatory responses" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0002543 brachyphalangia "abnormally short phalanges" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

 MP:0002754 dilated right ventricle "an expansion in the volume of the lower right chamber of the heart" [J:82859, pvb:Pierre Vanden Borre , Mouse Genome Informatics Curator]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0002764 short tibia " reduced length of the medial and larger bone of the lower leg" [J:12736, pvb:Pierre Vanden Borre , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

 MP:0002940 variable body spotting "the appearance of patches of white fur at random on the coat" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+,KitW/Kit+
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0002964 aortic elastic tissue lesions "focal pathological changes in the elastic lamella of the muscular layer of the main trunk of the arterial system" [RGD:Rat Genome Database submission, Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0002998 abnormal bone remodeling "aberrant process of the turnover of bone matrix that involves a balance of resorbtion and formation by osteoclasts and osteoblasts, respectively" [ava:Anna V. Anagnostopoulos , Mouse Genome Informatics Curator, MeSH:National Library of Medicine - Medical Subject Headings , 2003]
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Allelic Composition: Abl1tm2.1Goff/Abl1tm2.1Goff,Abl2tm1Ajk/Abl2tm1Ajk,Tg(Nes-cre)1Kln/0
Genetic Background: involves: 129 * C57BL/6 * C57BL/6J * SJL

 MP:0003084 abnormal skeletal muscle fiber morphology "anomalous structure of the skeletal muscle fibers, the large multinucleated cells that make up the skeletal muscles" [smb:Susan M. Bello, Mouse Genome Informatics Curator, Stedman s Medical Dictionary, 27th edition:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1tm1.2Lysa/Fbn1tm1.2Lysa
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

 MP:0003120 abnormal tracheal cartilage "any structural anomaly of the cartilaginous structures that support the trachea" [smb:Susan M. Bello, Mouse Genome Informatics Curator, J:97123]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0003140 dilated atria "an expansion in the volume of one or both of the upper chambers of the heart" [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0003141 cardiac fibrosis "formation of fibrous tissue within the heart often resulting from inflammation or injury " [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0003211 abnormal aorta elastic fiber morphology "malformation of a slender connective tissue fiber in aortic tissue characterized by great elasticity" [Stedman s Medical Dictionary , 27th edition:ISBN 0-683-40008-8, RGD:Rat Genome Database submission]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1.2Lysa/Fbn1tm1.2Lysa
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0003652 abnormal skin turgor "anomaly in the ability of the skin to resist deformation; influential factors include dehydration and age" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0003705 abnormal hypodermis morphology "malformed or atypical condition of the innermost and thickest layer of the skin composed of loose connective tissue containing adipoctyes" [monikat:Monika Tomczuk, Mouse Genome Informatics Curator]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0003725 increased autoantibody level "elevated level of antibodies to self-antigens present in the sera; often indicative of autoimmune disease" [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator, acv:Alicia Valenzuela, Genetic Resources Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,Il4ratm1Sz/Il4ra+
Genetic Background: involves: BALB/cJ * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0003905 abnormal aorta elastin content "anomaly in the physical amount of elastin in the aorta compared to the normal state" [RGD:Rat Genome Database submission]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/JKcc

Allelic Composition: Fbn1Tsk/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * C57BL/6J

 MP:0003920 abnormal right ventricle morphology "structural anomaly of the right lower chamber of the heart " [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0003921 abnormal left ventricle morphology "structural anomaly of the left lower chamber of the heart " [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0003923 abnormal left atrium morphology "structural anomaly of the left upper chamber of the heart " [csmith:Cynthia L. Smith , Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0003924 herniated diaphragm "protrusion of abdominal contents into the thoracic cavity through the diaphragm" [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0004003 abnormal vascular endothelial cell physiology "anomaly in the function of the cells that line the vasculature" [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: F11tm1Gjb/F11tm1Gjb
Genetic Background: B6.129X1-F11tm1Gjb

 MP:0004007 abnormal lung vasculature "malformation or disorganization of the blood vessels of the lung" [smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0004016 decreased bone mass "a reduction in the total amount of bone tissue contained in the skeleton" [monikat:Monika Tomczuk, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0004044 aortic dissection "a pathologic process, characterized by splitting of the media layer of the aorta, which leads to formation of a dissecting aneurysm" [anna:Anna Anagnostopoulos, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm1Hcd/Fbn1tm1Hcd
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0004127 thick hypodermis "increase in the width of the innermost and thickest layer of the skin composed of loose connective tissue containing adipoctyes" [anna:Anna Anagnostopoulos, Mouse Genome Informatics Curator]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0004355 short radius "reduced length of the short bone of the lateral forearm" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

 MP:0004359 short ulna "reduced length of the medial and larger of the two bones of the forearm" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

 MP:0004389 abnormal respiratory bronchiole morphology "any structural anomaly of the smallest bronchioles, which connect the terminal bronchioles to alveolar ducts and have alveoli rising from their walls" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition", MGI:brs "Beverly Richards-Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

 MP:0004509 abnormal pelvic girdle bone morphology "any structural anomaly of the bones of the pelvis by which the limbs attach to the axial skeleton " [MGI:cwg "Carroll-Ann W. Goldsmith, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0004593 long mandible "increased length of the lower bony framework of the mouth where the inferior teeth are held" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0004634 short metacarpal bones "reduced length of the five bones of the forepaws that articulate proximally with the carpal bones and distally with the phalanges" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

 MP:0004671 long ribs "increased length of the bones forming the bony wall of the chest" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0004686 decreased length of long bones "reduced end-to-end length of the several elongated bones of the extremities" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

 MP:0004695 increased length of long bones "greater end-to-end length of the several elongated bones of the extremities" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0004707 enlarged lumbar vertebrae "increased size of any or all of the six bony segments of the spine located anterior to the sacral vertebrae and posterior to the thoracic vertebrae" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0004735 enlarged thoracic cavity "increased size of the cavity in the vertebrate body enclosed by the ribcage between the diaphragm and the neck, and contains the lungs, heart, thoracic aorta, pulmonary artery and its branches, thymus gland, and the respiratory airway" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0004794 increased anti-nuclear antigen antibody level "elevated level of antibodies to nuclear antigens present in the sera" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0005014 increased B cell number "greater than normal B cell numbers" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0005048 thrombosis "formation within a tissue or the vascular lumen of a thrombus, an aggregation of coagulated blood containing platelets, fibrin, and entrapped cellular elements " [Pathology:ISBN 0-397-51047-0, cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0005081 abnormal stratum reticulare morphology "structural anomaly of the thicker, deeper layer of the dermis, containing blood and lymph vessles, nerves and nerve endings, and hair follicles" [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/JKcc

Allelic Composition: Fbn1Tsk/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * C57BL/6J

 MP:0005171 lack of coat pigmentation "fur is devoid of coloration and appears white " [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator, J:83500]
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Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

 MP:0005243 hemothorax "bleeding into the pleural space" [Pathology:ISBN 0-397-51047-0]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(CMV-cre)1Cgn/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * BALB/cJ * C57BL/6J

 MP:0005244 hemopericardium "bleeding into the pericardial space" [Pathology:ISBN 0-397-51047-0]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(CMV-cre)1Cgn/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * BALB/cJ * C57BL/6J

 MP:0005275 abnormal skin tensile strength "change from the normal maximum tension the skin can withstand without tearing" [Stedman s Medical Dictionary:ISBN 0-683-40008-8, J:84062]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0005501 abnormal skin physiology "atypical function of the membranous protective covering of the body" [cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator, Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0005503 abnormal tendon morphology "structural anomaly of the fibrous bands or cords of connective tissue at the ends of muscle fibers that attach muscles to bones and other structures" [MeSH:National Library of Medicine - Medical Subject Headings , 2003, J:45509]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm1.2Lysa/Fbn1tm1.2Lysa
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

 MP:0005508 abnormal skeleton morphology "malformation of the bony framework of the body in vertebrates" [Stedman s Medical Dictionary:ISBN 0-683-40008-8]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * C57BL/6 * CD-1

 MP:0005617 increased susceptibility to type IV hypersensitivity reaction "greater likelihood of developing an antigen-elicited cellular immune reaction that results in tissue damage, but does not require Ab participation; the response is seen at the area of contact " [Pathology:ISBN 0-397-51047-0, cwg:Carroll-Ann W. Goldsmith , Mouse Genome Informatics curator]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0006027 impaired alveologenesis "a block or reduction in the generation of alveoli" [smb:Susan M Bello, Mouse Genome Informatics Curator, J:85546]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0006120 mitral valve prolapse "collapse of one or more of the mitral valve cusps backward allowing blood to leak backwards, ususally the result of enlargement of one or more of the valve cusps" [ncbi:Matthew Mailman, NCBI request, smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0006133 arterial calcification "pathologic deposition of calcium salts in the arteries" [ncbi:Matthew Mailman, NCBI request, smb:Susan M Bello, Mouse Genome Informatics Curator]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0006278 aortic aneurysm "protruding sac formed by dilation of the aorta" [J:110586, MGI:smb "Susan M. Bello, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Hcd/Fbn1+,Tgfb2tm1Doe/Tgfb2+
Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0006432 abnormal costal cartilage morphology "any structural anomaly of the nonvascular, resilient, flexible connective tissue that connects the end of a true rib to the sternum or the end of a false rib with the costal cartilage above" [ISBN:0-8036-0655-9 "Taber s Cyclopedic Medical Dictionary", MGI:smb "Susan M. Bello, Mouse Genome Informatics Curator"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0008054 abnormal uterine NK cell morphology "any structural anomaly of the uterine subset of the non-T, non-B lymphocytes, having a granular morphology and which are important in innate immunity to viruses and other intracellular pathogens; in addition, these cells can kill certain tumor cells" [ISBN:0-8153-1691-7 "Immunobiology, The Immune System in Health and Disease"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0008097 increased plasma cell number "greater number of terminally differentiated, post-mitotic, short-lived cells of the B cell lineage devoted to producing large amounts of immunoglobulin" [CL:0000786, ISBN:0781735149, MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0008419 abnormal cutaneous microfibril morphology "any strucutral anomaly of the fiber-like strand of fibrillin that forms the scaffold of the cutaneous elastic fibers found in the extracellular matrix of the skin" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1tm1.2Lysa/Fbn1tm1.2Lysa
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

 MP:0008438 abnormal cutaneous collagen fibril morphology "any structural anomaly of the connective tissue bundles in the extracellular matrix of skin tissue that are composed of collagen, and play a role in skin strength and elasticity" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,Il4ratm1Sz/Il4ratm1Sz
Genetic Background: involves: BALB/cJ * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Tgfb1tm1N/Tgfb1+
Genetic Background: involves: 129S/SvEv * C57BL/6J * C57BL/10 * DBA/2

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0008568 abnormal interleukin secretion "anomaly in the production or cellular release of soluble factors which stimulate growth-related activities of leukocytes and other cell types, that can act to enhance cell proliferation and differentiation, DNA synthesis, secretion of other biologically active molecules and responses to immune and inflammatory stimuli" [MESH:D12.644.276.374.465]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0008672 increased interleukin-13 secretion "increase in the production or release of a cytokine synthesized by T-cells that produces proliferation, immunoglobulin isotype switching, and immunoglobulin production by immature B-cells, and appears to play a role in regulating inflammatory and immune responses" [MESH:D12.644.276.374.465.513]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0008700 decreased interleukin-4 secretion "reduction in the production or release of a soluble factor produced by activated T-cells that induces the expression of MHC class II genes and FC receptors on B-cells and causes their proliferation and differentiation; it also acts on T-cells, mast cells and several other hematopoietic lineage cells" [MESH:D12.644.276.374.465.504]
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Allelic Composition: Fbn1Tsk/Fbn1+,Stat6tm1Gru/Stat6tm1Gru
Genetic Background: involves: 129S2/SvPas * C57BL/10 * DBA/2

Allelic Composition: Fbn1Tsk/Fbn1+,Il4tm1Nnt/Il4tm1Nnt
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2

 MP:0008711 increased interleukin-9 secretion "increase in the production or release of a multifunctional cytokine secreted by primarily by activated TH2 cells that may play a role as a regulator of allergic inflammation, and has been shown to enhance the growth and differentiation of mast cells, and can act on a variety of other immune cells" [MESH:D12.644.276.374.465.509]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0008844 decreased subcutaneous adipose tissue amount "reduction in amount of adipose tissue beneath the skin" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm3.2Lysa/Fbn1tm3.2Lysa
Genetic Background: Not Specified

Allelic Composition: Fbn1tm3.2Lysa/Fbn1+
Genetic Background: Not Specified

Allelic Composition: Fbn1tm2.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

Allelic Composition: Fbn1tm3.1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0009346 decreased cancellous bone thickness "thinner than normal bone with a lattice-like or spongy structure" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0009545 abnormal dermis papillary layer "any structural anomaly of the more superficial, thin layer of the dermis that interdigitates with the epidermis" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0009826 abnormal dermis reticular layer collagen network "an anomaly in the amount or arrangement of the criss-crossing collagen fibers that form a strong elastic network normally arranged in parallel to the surface of the skin" [MGI:llw2 "Linda Washburn, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk +/+ Bloc1s6pa/JKcc

Allelic Composition: Fbn1Tsk/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * C57BL/6J

 MP:0009862 abnormal aorta elastic tissue morphology "any structural anomaly of the dense connective tissue which contains predominantly elastic fibers and is found in the tunica media of the aorta wall" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

 MP:0009866 abnormal aorta wall morphology "any structural anomaly of the part of the aorta that encloses the luminal space" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1Hcd/Fbn1+,Tgfb2tm1Doe/Tgfb2+
Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0009871 abnormal aorta tunica adventitia morphology "any structural anomaly of the outermost layer of the aorta wall, containing connective tissue and collagen and elastic fibers" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0009872 abnormal aorta tunica intima morphology "any structural anomaly of the innermost layer of the aorta, containing the endothelium and an inner elastic membrane" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0009873 abnormal aorta tunica media morphology "any structural anomaly of the middle layer of the aorta wall, containing the smooth muscle layer and elastic fibers" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz,Fbn2tm1Rmz/Fbn2tm1Rmz
Genetic Background: involves: 129

Allelic Composition: Fbn1tm3Rmz/Fbn1+,Fbn2tm1Rmz/Fbn2tm1Rmz
Genetic Background: involves: 129

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * CD-1

Allelic Composition: Fbn1tm1Lper/Fbn1+
Genetic Background: involves: 129/Sv * C57BL/6 * CD-1

 MP:0009932 skin fibrosis "invasion of fibrous connective tissue into the skin, often resulting from inflammation or injury" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

Allelic Composition: Fbn1Tsk/Fbn1+,KitW/Kit+
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

Allelic Composition: Fbn1tm3.1Hcd/Fbn1tm3.1Hcd
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0010139 aortitis "inflammation of the aorta" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

 MP:0010386 abnormal urinary bladder physiology "any functional anomaly of the distensible musculomembranous organ that serves to collect and store urine excreted by the kidneys" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010470 ascending aorta dilation "the luminal space of the ascending aorta is increased in volume or area, usually with an increase of contained fluid" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(CMV-cre)1Cgn/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * BALB/cJ * C57BL/6J

 MP:0010483 aortic sinus aneurysm "a protruding sac formed by the dilation of the wall of the aortic sinus resulting from a weakening of the vessel wall" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0010867 abnormal bone trabecula morphology "any structural anomaly of the network of intersecting plates and spicules in cancellous bone which form a meshwork of intercommunicating spaces filled with blood vessels and marrow; in mature bone, the trabeculae are aligned in parallel with the lines of major compressive or tensile force" [http://www.dorlands.com/ "Dorland s Illustrated Medical Dictionary, 31st edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0010869 decreased bone trabecula number "decreased number of intersecting plates and spicules in cancellous bone which form a meshwork of intercommunicating spaces filled with blood vessels and marrow; in mature bone, the trabeculae are aligned in parallel with the lines of major compressive or tensile force" [http://www.dorlands.com/ "Dorland s Illustrated Medical Dictionary, 31st edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0010876 decreased bone volume "reduced amount of space occupied by bone tissue in the skeleton" [MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm2Rmz/Fbn1tm2Rmz
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Prrx1-cre)1Cjt/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * SJL/J

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(Sp7-tTA,tetO-EGFP/cre)1Amc/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * C57BL/6J * CD-1

 MP:0010895 increased lung compliance "increased ability of the lung to distend in response to pressure without disruption, usually expressed as the unit volume of change in the lung per unit of pressure" [ISBN:070202788 "Saunders Comprehensive Veterinary Dictionary, 3rd edition"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010900 abnormal pulmonary interalveolar septum morphology "any structural anomaly of the tissue intervening between two adjacent pulmonary alveoli, which consists of the basement membranes of alveolar-lining epithelium (mostly type I pneumocytes) and capillary endothelium; thicker alveolar septal regions may also contain elastic fibers, collagen, interstitial cells, smooth muscle cells, mast cells, lymphocytes and monocytes" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition", MGI:csmith "Cynthia L. Smith, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010903 abnormal pulmonary alveolus wall morphology 
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010904 abnormal alveolar pore morphology "any structural anomaly of the openings in the alveolar septum that permit air flow between adjacent alveoli" [ISBN:0-683-40008-8 "Stedman s Medical Dictionary, 27th edition"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010908 dilated pulmonary alveolar ducts "expansion or widening of the lumens of the respiratory conducting tubes distal to the respiratory bronchiole that lead to the alveolar sacs and the alveoli" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010915 increased solitary pulmonary neuroendocrine cell number "increased number of the specialized non-ciliated, round- to oval-shaped epithelial cells found as solitary cells in the nasal respiratory epithelium and the mucosa of the larynx, trachea and bronchi up to the terminal bronchioles; PNECs are the first cell type to differentiate during early fetal lung development, produce amine (e.g. serotonin, 5-HT) and peptides (e.g. bombesin, calcitonin) with growth factor-like properties, and appear to be involved in processes ranging from lung development and respiratory physiology to repair, disease and carcinogenesis" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0010918 abnormal pulmonary neuroendocrine bodies "any structural anomaly of the corpuscular, organoid structures composed of PNECs, found as distinctive innervated clusters only within intrapulmonary airways, where they appear concentrated at airway branch points; NEBs reach from the basement membrane to the airway lumen and are thought to function as oxygen sensors" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0010919 increased number of pulmonary neuroendocrine bodies "greater number of the corpuscular, organoid structures composed of PNECs, found as distinctive innervated clusters only within intrapulmonary airways, where they appear concentrated at airway branch points; NEBs reach from the basement membrane to the airway lumen and are thought to function as oxygen sensors" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

 MP:0010922 alveolitis "inflammation of the small sac-like dilations of the distal airspace of the lung; acute pulmonary alveolitis involves exudate into the pulmonary alveoli and impaired gas exchange" [MGI:anna "Anna V. Anagnostopoulos, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010937 increased total lung capacity "greater volume of air contained in the lungs at the end of maximal inspiration" [MGI:csmith]
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Allelic Composition: Fbn1Tsk/Fbn1+
Genetic Background: B6.Cg-Fbn1Tsk

 MP:0010996 increased aorta wall thickness "increased depth of the part of the aorta that encloses the luminal space" [MGI:smb "Susan M. Bello, Mouse Genome Informatics Curator"]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0011085 complete postnatal lethality "premature death anytime between the neonatal period and weaning age of all organisms of a given genotype in a population (Mus: P1 to approximately 3 weeks of age)" [MGI:csmith]
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Allelic Composition: Fbn1tm1Rmz/Fbn1tm1Rmz
Genetic Background: involves: 129S4/SvJae * C57BL/6J

Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz
Genetic Background: Not Specified

Allelic Composition: Fbn1tm1Hcd/Fbn1tm1Hcd
Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Allelic Composition: Fbn1tm1.2Lysa/Fbn1tm1.2Lysa
Genetic Background: involves: C57BL/6

Allelic Composition: Fbn1tm1Lper/Fbn1tm1Lper
Genetic Background: involves: 129/Sv * CD-1

Allelic Composition: Fbn1tm1Itl/Fbn1tm1Itl
Genetic Background: involves: 129S1/Sv * 129X1/SvJ

Allelic Composition: Fbn1tm1.1Itl/Fbn1tm3Rmz,Tg(CMV-cre)1Cgn/0
Genetic Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ * BALB/cJ * C57BL/6J

 MP:0011088 partial neonatal lethality "the appearance of lower than Mendelian ratios of organisms of a given genotype due to death of some, but not all of the organisms within the neonatal period after birth (Mus: P0)" [MGI:csmith]
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Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

 MP:0011092 complete embryonic lethality "death of all organisms of a given genotype in a population within the embryonic period prior to organogenesis (Mus: prior to E14)" [MGI:csmith]
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Allelic Composition: Fbn1tm2.1Hcd/Fbn1+,Itgb3tm1Hyn/Itgb3+
Genetic Background: involves: 129S * 129X1/SvJ * C57BL/6J * FVB/N

 MP:0011096 complete embryonic lethality before somite formation "death of all organisms of a given genotype in a population between the point of implantation and somite formation (Mus: E4.5 to less than E8)" [MGI:csmith]
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Allelic Composition: Foxe3tm1Mjam/Foxe3tm1Mjam
Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

 MP:0011098 complete embryonic lethality during organogenesis "death of all organisms of a given genotype in a population between embryo turning and the completion of organogenesis (Mus: E9 to less than E14)" [MGI:csmith]
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Allelic Composition: Fbn1tm1Lper/Fbn1tm1Lper
Genetic Background: involves: 129/Sv * CD-1

 MP:0011099 complete lethality throughout fetal growth and development "death of all organisms of a given genotype in a population between the completion of organogenesis and birth (Mus: E14 to approximately E18.5)" [MGI:csmith]
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Allelic Composition: Fbn1tm3Rmz/Fbn1tm3Rmz,Fbn2tm1Rmz/Fbn2tm1Rmz
Genetic Background: involves: 129

 MP:0011101 partial prenatal lethality "the appearance of lower than Mendelian ratios of organisms of a given genotype due to death of some, but not all of the organisms between fertilization and birth (Mus: approximately E18.5)" [MGI:csmith]
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Allelic Composition: Fbn1tm1.2Lysa/Fbn1+
Genetic Background: involves: C57BL/6

 MP:0011108 partial embryonic lethality during organogenesis "the appearance of lower than Mendelian ratios of organisms of a given genotype due to death of some, but not all of the organisms between embryo turning and the completion of organogenesis (Mus: E9 to less than E14)" [MGI:csmith]
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Allelic Composition: Fbn1tm1Lper/Fbn1tm1Lper
Genetic Background: involves: 129/Sv * CD-1

 MP:0011109 partial lethality throughout fetal growth and development "the appearance of lower than Mendelian ratios of organisms of a given genotype due to death of some, but not all of the organisms between the completion of organogenesis and birth (Mus: E14 to approximately E18.5)" [MGI:csmith]
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Allelic Composition: Fbn1tm3Rmz/Fbn1+,Fbn2tm1Rmz/Fbn2tm1Rmz
Genetic Background: involves: 129

 MP:0011575 dilated aorta bulb "the luminal space of the aorta bulb is increased in volume or area, usually with an increase of contained fluid" [MGI:csmith]
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Allelic Composition: Fbn1tm1Hcd/Fbn1+,Tgfb2tm1Doe/Tgfb2+
Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J

 MP:0011696 absent mast cells "any structural anomaly of a cell that is found in almost all tissues containing numerous basophilic granules and capable of releasing large amounts of histamine and heparin upon activation; progenitors leave bone marrow and mature in connective and mucosal tissue; mature mast cells are found in all tissues, except the bloodstream." [CL:0000097]
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Allelic Composition: Fbn1Tsk/Fbn1+,KitW/KitW-v
Genetic Background: involves: C57BL/6 * C57BL/10 * DBA/2 * WB/ReJ

  


Interacting proteins (from Reactome)
Interactor ID Name Interaction type
 ENSMUSG00000049723 Mmp12 / P34960 / Macrophage metalloelastase / P39900* / matrix metallopeptidase 12*  / reaction
 ENSMUSG00000017737 Mmp9 / P41245 / Matrix metalloproteinase-9 / P14780* / matrix metallopeptidase 9*  / reaction
 ENSMUSG00000029675 Eln / P54320 / Elastin / P15502*  / complex
 ENSMUSG00000050578 Mmp13 / P33435 / Collagenase 3 / P45452* / matrix metallopeptidase 13*  / reaction
 ENSMUSG00000020522 Mfap3 / microfibril associated protein 3 / P55082*  / reaction / complex
 ENSMUSG00000020689 Itgb3 / O54890 / Integrin beta-3 / P05106* / AC068234.1* / integrin subunit beta 3*  / reaction / complex
 ENSMUSG00000017344 Vtn / P29788 / vitronectin / P04004*  / complex / reaction
 ENSMUSG00000024529 Lox / P28301 / Protein-lysine 6-oxidase / P28300* / lysyl oxidase*  / reaction
 ENSMUSG00000021186 Fbln5 / Q9WVH9 / Fibulin-5 / Q9UBX5*  / complex
 ENSMUSG00000027087 Itgav / P43406 / Integrin alpha-V Integrin alpha-V heavy chain Integrin alpha-V light chain / P06756* / integrin subunit alpha V*  / complex / reaction
 ENSMUSG00000030116 Mfap5 / Q9QZJ6 / Microfibrillar-associated protein 5 / Q13361* / microfibril associated protein 5*  / complex / reaction
 ENSMUSG00000026971 Itgb6 / Q9Z0T9 / Integrin beta-6 / P18564* / integrin subunit beta 6*  / complex / reaction
 ENSMUSG00000040488 Ltbp4 / Q8K4G1 / Latent-transforming growth factor beta-binding protein 4 / Q8N2S1*  / reaction / complex
 ENSMUSG00000027204 Fbn1 / Q61554 / Fibrillin-1 Asprosin / P35555* / fibrillin 1*  / -
 ENSMUSG00000025809 Itgb1 / P09055 / Integrin beta-1 / P05556* / integrin subunit beta 1*  / complex / reaction
 ENSMUSG00000000555 Itga5 / P11688 / Integrin alpha-5 Integrin alpha-5 heavy chain Integrin alpha-5 light chain / P08648* / integrin subunit alpha 5*  / complex / reaction
 ENSMUSG00000001870 Ltbp1 / Q8CG19 / Latent-transforming growth factor beta-binding protein 1 / Q14766*  / complex / reaction
 ENSMUSG00000029163 Q99K41 / Emilin1 / Q9Y6C2* / elastin microfibril interfacer 1*  / complex
 ENSMUSG00000042436 Mfap4 / Q9D1H9 / Microfibril-associated glycoprotein 4 / P55083* / microfibril associated protein 4*  / reaction / complex
 ENSMUSG00000031740 Mmp2 / P33434 / 72 kDa type IV collagenase PEX / P08253* / matrix metallopeptidase 2*  / reaction
 ENSMUSG00000060572 Mfap2 / microfibril associated protein 2 / P55001*  / complex / reaction
 ENSMUSG00000032334 Loxl1 / lysyl oxidase like 1 / Q08397*  / reaction
 ENSMUSG00000048222 C0HKD9 / C0HKD8 / Mfap1b / Microfibrillar-associated protein 1A / MFAP1* / P55081* / microfibril associated protein 1*  / reaction / complex
 ENSMUSG00000030530 Furin / P23188 / P09958* / furin, paired basic amino acid cleaving enzyme*  / reaction






 

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