Article
Fibulin-3 knockout mice demonstrate corneal dysfunction but maintain normal retinal integrity.
Journal of molecular medicine (Berlin, Germany) - 1 Nov 2020
Daniel Steffi, Renwick Marian, Chau Viet Q, Datta Shyamtanu, Maddineni Prabhavathi, Zode Gulab, Wade Emma M, Robertson Stephen P, Petroll W Matthew, Hulleman John D
Abstract excerpt
Fibulin-3 (F3) is an extracellular matrix glycoprotein found in basement membranes across the body. An autosomal dominant R345W mutation in F3 causes a macular dystrophy resembling dry age-related macular degeneration (AMD), whereas genetic removal of wild-type (WT) F3 protects mice from sub-retinal pigment epithelium (RPE) deposit formation. These observations suggest that F3 is a protein which can regulate...
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