Xin actin-binding repeat-containing protein 2 (XIRP2)

Xin actin-binding repeat-containing protein 2 (XIRP2) is a cytoskeletal protein involved in the binding and protection of actin filaments from depolymerization[8][3]. XIRP2 is localized predominantly in striated muscle (including cardiac muscle), where it plays a critical scaffolding role in the formation and maturation of intercalated disks that connect cardiac myocytes and regulate their electrophysiological properties[6][8][4]. In the inner ear, XIRP2 is enriched in sensory hair cells, with specialized isoforms responsible for maintaining stereocilia structure, facilitating repair after mechanical damage, and preserving hearing function[1][2]. Deficiency or mutation of XIRP2 leads to pathology such as cardiac conduction defects, arrhythmias, cardiomyopathy, and hearing loss, and is suspected in certain neurodevelopmental disorders[3][1][4]. XIRP2 is not currently a therapeutic target for any approved drug, but genetic defects may have diagnostic value in specific diseases.

Other names

CMYA3Beta-xinCardiomyopathy-associated protein 3XeplinMyomaxinXin repeat protein 2