Nebulette (NEBL)

Nebulette is a cardiac-specific protein in the nebulin family, encoded by the NEBL gene. It is primarily expressed in cardiac muscle, where it binds actin and interacts with thin filaments and Z-line–associated proteins in striated muscle. Nebulette is essential for organizing and maintaining the structural integrity of myofibrils, especially at the Z-disk, and is involved in the assembly and stabilization of cardiac myofibrils[1][3]. It interacts with key cytoskeletal and focal adhesion proteins including zyxin, filamin C, Xin, α-actinin, tropomyosin, troponin T, and myopalladin[2][1]. Alternative splicing results in multiple isoforms, including LIM-nebulette, which is found in non-muscle cells and may be a component of focal adhesion complexes[3]. Mutations in NEBL result in various cardiomyopathies and other pathologies, highlighting its critical role in cardiac biology and disease[2][1][3].

Other names

NebuletteNEBLC10orf113LIM-nebuletteLASP2LNEBLbA165O3.1LIM and SH3 protein 2actin-binding Z-disk protein