Syntrophin, alpha 1 Protein

Background

Alpha-1-syntrophin (SNTA1) is a modular adaptor protein that interacts with members of the dystrophin-associated glycoprotein complex (DGC), linking the cytoskeleton to membrane-bound signaling proteins. It contains PDZ, PH, and syntrophin-specific domains that facilitate interactions with signaling molecules such as ion channels, nitric oxide synthase (NOS), and kinases, enabling the coordination of signal transduction pathways involved in membrane stability, ion homeostasis, and cellular signaling. SNTA1 is widely expressed in skeletal and cardiac muscle, where it regulates membrane integrity, ion transport, and nitric oxide signaling, critical for muscle contraction and vascular function. Dysregulation of SNTA1 is linked to muscular dystrophies, cardiac arrhythmias, and cancers due to its role in maintaining cytoskeletal organization and signaling balance. Mutations in SNTA1 are associated with long QT syndrome, a condition characterized by cardiac arrhythmias and impaired ion channel regulation. Functional studies highlight its essential role in maintaining cellular signaling, membrane structure, and tissue homeostasis under physiological and pathological conditions.