The gene NEBL (nebulette) encodes a nebulin like protein having a molar mass of 107kDa. It is predominantly expressed in the cardiac muscle. The protein is very similar to C-terminal region of human nebulin, an actin-binding skeletal muscle protein. They share a family of highly related C-terminal repeats, a serine-rich domain, and an SH3 domain. The gene is mapped to human chromosome 10p12. A short linker domain links the 22 nebulin-like repeats to a C-terminal Src homology 3 (SH3) domain. The repeat domain binds to actin filaments and the linker domain targets this association to Z-lines and dense bodies. The linker and SH3 domains are capable of differentiating between dense bodies and Z-lines. The alternative splice variant called LIM-nebulette is expressed in non-muscle cells. It interacts with Zyxin via the SH3 domain along with Lasp-1 and functions in the organization of focal adhesions.
Synonyms: Anti-Actin-binding Z-disk protein; Anti-Nebulette
Storage: -20C
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Biochem Physiol Actions: The protein, nebulette, encoded by the gene NEBL is found to be expressed aligned with the nascent myofibrils early during myofibrillogenesis. It may function in myofibril organization and function. It also interacts with I-Z-I complex of cardiac myofibrils and functions in the assembly of the Z-line.
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