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货号： bs-9468R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9468R-Bio
- 英文名称
- Anti-Nebulette/Biotin
- 中文名称
- 生物素标记的肌动蛋白结合蛋白Z盘状蛋白抗体
- 别 名
- Actin binding Z disk protein; bA56H7.1; LIM nebulette; LNEBL; NEBL; NEBL_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 116kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Nebulette
- 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.
- 产品介绍
- background:
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.
Database links:Entrez Gene: 10529 Human
Entrez Gene: 74103 Mouse
Omim: 605491 Human
SwissProt: O76041 Human
SwissProt: Q9DC07 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.