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Different fibrillin-1 mutant mice
ORGANISM(S): Mus musculus 
Different fibrillin-1 mutant mice Keywords: other
ORGANISM(S): Mus musculus 
2004-10-20 | GSE1852 | GEO
Fibrillin-1, an extracellular matrix (ECM) protein encoded by FBN1 gene, serves as the microfibril scaffold crucial for elastic fiber formation and homeostasis in pliable tissue such as the skin. Besides causing Marfan syndrome, some mutations in FBN1 result in scleroderma, marked by hardened and th...
ORGANISM(S): Mus Musculus (mouse) 
Fibrillin-1 deficient mice
Fibrillins are the major components of microfibrils in the extracellular matrix of elastic and non-elastic tissues. Fibrillin-1 contains one evolutionarily conserved Arg-Gly-Asp (RGD) sequence which mediates cell-matrix interactions through cell-surface integrins. Mutations in close vicinity to the ...
ORGANISM(S): synthetic construct Homo sapiens 
2018-12-10 | GSE82083 | GEO
Fibrillins are the major components of microfibrils in the extracellular matrix of elastic and non-elastic tissues. Fibrillin-1 contains one evolutionarily conserved Arg-Gly-Asp (RGD) sequence which mediates cell-matrix interactions through cell-surface integrins. Mutations in close vicinity to the ...
ORGANISM(S): Homo sapiens 
2018-12-10 | GSE82079 | GEO
Mutations in the fibrillin-1 gene result in cardiovascular, ocular, and skeletal abnormalities in Marfan syndrome. Here we show that a fibrillin-1 mutation also alters the neovessel formation. In the mouse retina vascularization model, fibrillin-1 is detected at the basement membrane underlying the ...
ORGANISM(S): Homo sapiens (Human) 
2023-05-31 | PXD029834 | Pride
Fibrillin-1 (FBN1) is the major component of extracellular matrix microfibrils, which are required for proper development of elastic tissues including heart and lung. Through protein-protein interactions with latent TGF-beta binding protein 1 (LTBP1), microfibrils assist regulation of TGF-beta signa...
ORGANISM(S): Homo sapiens (Human) 
2021-08-06 | PXD025725 | Pride
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