生物素蛋白连接酶抗体
规格：1mg/1ml
英文名： HLCS
别名： Biotin [acetyl CoA carboxylase] ligase; Biotin [methylcrotonoyl CoA carboxylase] ligase; Biotin [methylmalonyl CoA carboxytransferase] ligase; Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] liga
分子量： 80kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HLCS
交叉反应：Human, Mouse, Dog,
细胞定位：细胞浆
产生物素蛋白连接酶抗体品介绍：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,生物素蛋白连接酶抗体 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.生物素蛋白连接酶抗体 Clinical and biochemical symptoms improve dramatically with administration of biotin. Similarity: Belongs to the biotin--protein ligase family. Gene ID: 3141 Database links: Entrez Gene: 3141 Human Entrez Gene: 110948 Mouse Entrez Gene: 288240 Rat 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.
生物素蛋白连接酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid