Fibrillin-1 Regulates Arteriole Integrity in the Retina.

ABSTRACT: Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues. Mutations in the fibrillin-1 gene are associated with cardiovascular, ocular, and skeletal abnormalities in Marfan syndrome. Fibrillin-1 is a component of the wall of large arteries but has been poorly described in other vessels. We examined the microvasculature in the retina using wild type mice and two models of Marfan syndrome, Fbn1^C1041G/+ and Fbn1^mgR/mgR. In the mouse retina, fibrillin-1 was detected around arterioles, in close contact with the basement membrane, where it colocalized with MAGP1. Both a mutation in fibrillin-1 or fibrillin-1 underexpression characteristically altered the microvascul

SUBMITTER: Alonso F 

PROVIDER: S-EPMC9599515 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature