fibrilinas1

Fibrilinas1 is a secreted extracellular matrix protein implicated in the organization and stabilization of connective tissue microfibrils. It is described as a member of the fibrillin-like family and shares the modular architecture common to fibrillins, including calcium-binding repeats and disulfide-rich domains that support folding and intermolecular interactions.

The protein is predicted to contain a signal peptide for secretion and a series of extracellular matrix–associated repeats, consistent with localization to the extracellular matrix. Its size and domain composition suggest a role in assembling or maintaining filamentous ECM structures that provide tissue elasticity.

Fibrilinas1 is encoded by the FIB1 gene. Expression has been detected in multiple connective tissues in vertebrate species, with higher apparent levels in skin, vasculature, and sclera. In developmental studies, the transcript is enriched during periods of extracellular matrix remodeling.

Functional studies propose involvement in microfibril assembly and maintenance, potentially through interactions with canonical fibrillins and other ECM adapters such as fibulins. Some data also hint at a role in modulating signaling pathways linked to the latent TGF-β complex, a function hypothesized for several fibrillin-family members.

Clinical significance remains unsettled. No disease has been definitively linked to mutations in fibrilinas1, and reported associations are preliminary. The protein is a focus of ongoing research, with resources including expression profiles, antibody reagents, and model-system studies described in current literature.