Nebulin-related-anchoring protein (NRAP)

Nebulin-related-anchoring protein (NRAP) is a muscle-specific cytoskeletal protein of approximately 1,730 amino acids (~197 kDa), encoded by the NRAP gene on chromosome 10q25.3. It contains a cysteine-rich LIM domain, 11 simple repeats, and 5 super repeats, facilitating interactions with α-actin, talin, actin, vinculin, KLHL41, and muscle LIM protein. NRAP is essential for myofibrillar assembly and force transmission, anchoring terminal actin filaments to the cell membrane at cardiac intercalated discs and skeletal muscle myotendinous junctions. Mutations in NRAP have been identified as causative in rare autosomal recessive forms of cardiomyopathy, notably dilated cardiomyopathy and left ventricular non-compaction cardiomyopathy, due to loss-of-function effects that impair myofibrillar structure and function. While NRAP is critical for muscle architecture, it is not currently considered a direct therapeutic target, and no specific drugs interact with this protein.

Other names

NRAPN-RAPNebulin-related anchoring proteinnebulin related anchoring proteinnebulin-related-anchoring protein