Description | Nebulette is a cardiac-specific protein that localizes to the Z-disc [ ]. It interacts with tropomyosin and is important in stabilizing actin thin filaments in cardiac muscles []. Polymorphisms in the nebulette gene are associated with dilated cardiomyopathy, with some mutations resulting in severe heart failure []. Nebulette is a 107kDa protein that contains an N-terminal acidic region, multiple nebulin repeats, and a C-terminal SH3 domain. LIM-nebulette, also called Lasp2 (LIM and SH3 domain protein 2), is an alternatively spliced variant of nebulette. Although it shares a gene with nebulette, Lasp2 is not transcribed from a muscle-specific promoter, giving rise to its multiple tissue expression pattern with highest amounts in the brain [, ]. It can crosslink actin filaments and it affects cell spreading []. Lasp2 is a 34kDa protein containing an N-terminal LIM domain, three nebulin repeats, and a C-terminal SH3 domain. | Name | Nebulette, SH3 domain |
Short Name | Nebulette_SH3 | Type | Domain |