anti-Alpha Cardiac Actin, Rabbit Monoclonal (RM257)

Actins are a family of globular multi-functional proteins that form microfilaments and are important for cell movement and the tensing (contraction) of muscles. ACTC1 (Cardiac alpha actin) together with regulatory proteins tropomyosin and 3 troponins (C, I, and T) forms the thin contractile filament, in turn connecting the myocellular Z-disc with the thick filament protein myosin. Polymerization of globular actin (G-actin) leads to a structural filament (F-actin) in the form of a two-stranded helix. Each actin can bind to four others. The protein encoded by this gene belongs to the actin family which is comprised of three main groups of actin isoforms, alpha, beta and gamma. The alpha actins are found in muscle tissues and are a major constituent of the contractile apparatus. Defects in this gene have been associated with idiopathic dilated cardiomyopathy (IDC) and familial hypertrophic cardiomyopathy (FHC). Mutations in ACTC1 are associated in autosomal dominant heart failure and familial atrial septal defect (ASD). Cardiac (ACTC1) and skeletal (ACTA1) alpha actins differ by only four amino acids (Asp4Glu, Glu5Asp, Leu301Met, Ser360Thr; cardiac/skeletal).