NRAP Recombinant Rabbit mAb (S-4533-5)

NRAP (Nebulin-related anchoring protein) is a large sarcomeric protein specifically expressed in striated muscle (skeletal and cardiac muscle), encoded by the NRAP gene located on human chromosome 10q25.3, containing approximately 1,731 amino acids with a theoretical molecular weight of about 197 kDa. Its structure consists of an N-terminal LIM zinc-binding domain and a C-terminal actin-binding domain containing multiple nebulin repeats, with the C-terminal region comprising approximately 44 nebulin repeats. The functional localization of NRAP is highly specific: it is enriched at the myotendinous junction in skeletal muscle and localizes to the intercalated disc in cardiac muscle. It is thought to play a key role in sarcomere assembly and mechanical signal transduction by anchoring actin filaments at the ends of myofibrils to submembranous protein complexes and transmitting myofibrillar tension to the extracellular matrix. Its interaction network includes cytoskeletal and adhesion-related proteins such as actin, α-actinin, KLHL41, TLN1, and VCL. Clinically, biallelic loss-of-function mutations in the NRAP gene have been demonstrated to cause recessive dilated cardiomyopathy, highlighting its importance in the maintenance of myocardial structure and function.