FLNB

Filamin B (FLNB), encoded by the FLNB gene, is an actin-binding protein also known as FLN2 or ABP-280L. It cross-links actin filaments into dynamic three-dimensional networks, stabilizing the cytoskeleton and mediating interactions between actin and membrane proteins (e.g., integrins) or signaling molecules, supporting cell motility and mechanical stress responses.
FLNB participates in integrin and TGF-β signaling pathways, regulating cell differentiation and extracellular matrix remodeling. Pathogenic mutations cause skeletal dysplasias like Larsen syndrome, atelosteogenesis types I/III, and spondylocarpotarsal synostosis. No approved drugs target FLNB directly, but preclinical studies explore strategies to correct mutation-induced cytoskeletal dysfunction for treating these genetic skeletal disorders.