| 产品编号 | bs-18044R-AF647 |
| 英文名称 | Anti-HLCS/AF647 |
| 中文名称 | AF647标记的生物素蛋白连接酶抗体 |
| 别 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 免疫学 信号转导 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human, Mouse, Dog, ) |
| 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 | 80kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HLCS |
| 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 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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
