FITC标记的Tafazzin蛋白抗体
产品名称： FITC标记的Tafazzin蛋白抗体
英文名称： Anti-Tafazzin/FITC
产品编号： HZ-15504R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Tafazzin/FITC Conjugated antibody
FITC标记的Tafazzin蛋白抗体
| 英文名称 | Anti-Tafazzin/FITC |
| 中文名称 | FITC标记的Tafazzin蛋白抗体 |
| 别 名 | Barth syndrome; Cardiomyopathy dilated 3A (X linked); EFE2; Endocardial fibroelastosis 2; Protein G4.5; Tafazzin; TAZ; TAZ protein; TAZ protein; TAZ_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 信号转导 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Cow, Sheep, |
| 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 | 33kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Tafazzin |
| 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 protein that is expressed at high levels in cardiac and skeletal muscle. Mutations in this gene have been associated with a number of clinical disorders including Barth syndrome, dilated cardiomyopathy (DCM), hypertrophic DCM, endocardial fibroelastosis, and left ventricular noncompaction (LVNC). Multiple transcript variants encoding different isoforms have been described. A long form and a short form of each of these isoforms is produced; the short form lacks a hydrophobic leader sequence and may exist as a cytoplasmic protein rather than being membrane-bound. Other alternatively spliced transcripts have been described but the full-length nature of all these transcripts is not known. [provided by RefSeq, Jul 2008]. Function: Some isoforms may be involved in cardiolipin (CL) metabolism. Subcellular Location: Isoform 1, 3, 4, 5, 7: Membrane; Single-pass membrane protein. Isoform 2, 6, 8, 9: Cytoplasm (Probable). Tissue Specificity: High levels in cardiac and skeletal muscle. Up to 10 isoforms can be present in different amounts in different tissues. Most isoforms are ubiquitous. Isoforms that lack the N-terminus are found in leukocytes and fibroblasts, but not in heart and skeletal muscle. Some forms appear restricted to cardiac and skeletal muscle or to leukocytes. DISEASE: Barth syndrome (BTHS) [MIM:302060]: An X-linked disease characterized by dilated cardiomyopathy with endocardial fibroelastosis, a predominantly proximal skeletal myopathy, growth retardation, neutropenia, and organic aciduria, particularly excess of 3-methylglutaconic acid. Additional features include hypertrophic cardiomyopathy, isolated left ventricular non-compaction, ventricular arrhythmia, motor delay, poor appetite, fatigue and exercise intolerance, hypoglycemia, lactic acidosis, hyperammonemia, and dramatic late catch-up growth after growth delay throughout childhood. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the taffazin family. Database links: Entrez Gene: 6901 Human Entrez Gene: 66826 Mouse Omim: 300394 Human SwissProt: Q16635 Human Unigene: 409911 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码在心脏和骨骼肌中高水平表达的蛋白质。该基因的突变与许多临床病症相关，包括Barth综合征、扩张型心肌病（DCM）、肥厚性DCM、心内膜弹力纤维增生症和左心室致密化不全（LVNC）。已经描述了编码不同亚型的多个转录变体。每个形态的长形式和短形式被产生；短形式缺少疏水性前导序列，并且可以作为细胞质蛋白而不是膜结合存在。其他交替剪接转录本已被描述，但所有这些转录本的全长性质尚不清楚。[ RefSeq，JUL 2008 ]提供。