| 產(chǎn)品編號(hào) | bs-15504R |
| 英文名稱(chēng) | Tafazzin Rabbit pAb |
| 中文名稱(chēng) | Tafazzin蛋白抗體 |
| 別 名 | Barth syndrome; Cardiomyopathy dilated 3A(X linked); EFE2; Endocardial fibroelastosis 2; Protein G4.5; Tafazzin; TAZ; TAZ protein; TAZ protein; TAZ_HUMAN. |
| 研究領(lǐng)域 | 腫瘤 信號(hào)轉(zhuǎn)導(dǎo) 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Sheep,Cow,Dog) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 33 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Tafazzin: 201-292/292 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: Q16635 Gene ID: 6901 Database links: Entrez Gene: 6901 Human Entrez Gene: 66826 Mouse Omim: 300394 Human SwissProt: Q16635 Human Unigene: 409911 Human |
