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货号: bs-19222R-PE-Cy3 基本售价: 2980.0 元 规格: 100ul
产品信息
- 产品编号
- bs-19222R-PE-Cy3
- 英文名称
- Anti-Nexilin/PE-Cy3
- 中文名称
- PE-Cy3标记的F-肌动蛋白结合蛋白抗体
- 别 名
- 1110046H09Rik; AA553326; F-actin binding protein; F-actin-binding protein; MGC104234; MGC138865; MGC138866; Nelin; Nexilin; Nexn; NEXN_HUMAN; nF actin binding protein; OTTHUMP00000038336.
- 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 细胞生物 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,
- 产品应用
- 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
- 81kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Nexilin
- 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:
This gene encodes a filamentous actin-binding protein that may function in cell adhesion and migration. Mutations in this gene have been associated with dilated cardiomyopathy, also known as CMD1CC. Alternatively spliced transcript variants have been described.[provided by RefSeq, Feb 2010]
Function:
Involved in regulating cell migration through association with the actin cytoskeleton. Has an essential role in the maintenance of Z line and sarcomere integrity.
Subcellular Location:
Cytoplasm; cytoskeleton. Cell junction; adherens junction. Cytoplasm; myofibril > sarcomere; Z line. Localizes to the cell-matrix AJ. Not found at the cell-cell AJ.
Tissue Specificity:
Abundantly expressed in heart and skeletal muscle, and at lower levels in placenta, lung, liver and pancreas. Also expressed in HeLa S3 and Molt-4 cells.
DISEASE:
Defects in NEXN are the cause of cardiomyopathy dilated type 1CC (CMD1CC) [MIM:613122]. A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in NEXN are the cause of cardiomyopathy familial hypertrophic type 20 (CMH20) [MIM:613876]. CMH20 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Similarity:
Contains 1 Ig-like (immunoglobulin-like) domain.
Database links:Entrez Gene: 91624Human
Entrez Gene: 68810Mouse
Entrez Gene: 246172Rat
Omim: 613121Human
SwissProt: Q0ZGT2Human
SwissProt: Q7TPW1Mouse
SwissProt: Q9Z2J4Rat
Unigene: 612385Human
Unigene: 200188Mouse
Unigene: 107975Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.