重组Nebulette蛋白(NEBL)
交货期:1周
产品别名:重组肌动蛋白结合蛋白Z盘状蛋白
Recombinant NEBL
基因名:
NEBL
产品别名:
C10orf113; LASP2; LNEBL; bA165O3.1; NEBL; nebulette; nebulette; nebulette; LIM and SH3 protein 2; LIM-nebulette; actin-binding Z-disk protein; Nebulette蛋白(NEBL); 肌动蛋白结合蛋白Z盘状蛋白;
背景信息:
Nebulette binds to actin and plays an important role in Z-disk assembly. It is a cardiac homolog of nebulin and its expression is abundant in cardiac muscle. Nebulette defects are assocated with non-familial idiopathic cardiacmyopathy.
Nebulin and nebulette are homologous thin-filament associated proteins expressed in skeletal and cardiac muscles, respectively. Nebulette, unlike nebulin, is confined to the Z-disc region of the sarcomere and does not span the whole thin filament length. Nebulette colocalizes with α-actinin in the pre-, nascent, and mature myofibrils. A polymorphism in the actin-binding motif of nebulette is a genetic marker of susceptibility to nonfamilial idiopathic dilated cardiomyopathy (IDC), characterized by a thin-walled heart and systolic dysfunction.
产品发布日期:2026.03.20