| 產(chǎn)品編號(hào) | bs-18044R |
| 英文名稱 | Rabbit Anti-HLCS antibody |
| 中文名稱 | 生物素蛋白連接酶抗體 |
| 別 名 | Biotin [acetyl CoA carboxylase] ligase; Biotin [methylcrotonoyl CoA carboxylase] ligase; Biotin [methylmalonyl CoA carboxytransferase] ligase; Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase; Biotin apo protein ligase; Biotin apo-protein ligase; Biotin protein ligase; Biotin--[acetyl-CoA-carboxylase] ligase; BPL1_HUMAN; HCS; HLCS; Holocarboxylase synthetase; Holocarboxylase synthetase, EC 6.3.4. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Dog) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,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 | 80kDa |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HLCS: 221-320/726 |
| 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 an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011] Function: Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase. Subcellular Location: Cytoplasm. Mitochondrion. Tissue Specificity: Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung. DISEASE: Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin. Similarity: Belongs to the biotin--protein ligase family. SWISS: P50747 Gene ID: 3141 Database links: Entrez Gene: 3141 Human Entrez Gene: 110948 Mouse Omim: 609018 Human SwissProt: P50747 Human SwissProt: Q920N2 Mouse Unigene: 371350 Human Unigene: 30921 Mouse |
