产品货号 :  mlR9468

英文名称 :  Nebulette

中文名称 :  肌动蛋白结合蛋白Z盘状蛋白抗体

别    名 :  Actin binding Z disk protein; bA56H7.1; LIM nebulette; LNEBL; NEBL; NEBL_HUMAN.  

研究领域 :  心血管  信号转导  结合蛋白  细胞骨架  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :   Human, Mouse, Rat,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  116kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human Nebulette:321-420/1014

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

产品介绍 :   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.

Function:

Binds to actin and plays an important role in the assembly of the Z-disk. Isoform 2 might play a role in the assembly of focal adhesion.

Subunit:

Isoform 2 interacts with ZYX (Zyxin).

Subcellular Location:

Cytoplasmic.

Tissue Specificity:

Abundantly expressed in cardiac muscle, but not in skeletal or smooth muscle. Localized to Z-lines in cardiac cells and to dense bodies in nonmuscle cells. Isoform 2 is expressed in non-muscle cells such as in fibroblasts.

Similarity:

Contains 23 nebulin repeats.

Contains 1 SH3 domain.

SWISS:

O76041

Gene ID:

10529

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片