生物素酶抗体 BTD
产品简介
生物素酶抗体应用于IHC、WB、 IF、IP、ELISA等科研实验,按理化性质和生物学功能IgM、IgG、IgA、IgE、IgD五类。按抗体的来源,可将其分为天然抗体和**抗体。生物素酶抗体生产每个流程都执行严格的检测标准,保证蛋白抗原产品质量,质量稳定,实验效果明显。
产品详细信息
生物素酶抗体
规格:1mg/1ml
英文名: BTD
别名: Biotinase; Biotinidase; Btd; Sp8; BTD_HUMAN; EC 3.5.1.12.
分子量: 57kDa
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 Biotinid
交叉反应:Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep,
细胞定位:分泌型蛋白
生物素酶抗体产品介绍:background: Biotin, also known as vitamin B7, is an essential water-soluble vitamin that is a cofactor in glucogenesis and in the metabolism of fatty acids and leucine. Biotinidase is a 523 amino acid enzyme that catalyzes the hydrolysis of biocytin to biotin and lysine. Secreted into extracellular space, biotinidase is expressed in liver, heart, placenta, brain, skeletal muscle, pancreas and kidney. Biotinidase contains one carbon-nitrogen hydrolase domain, which is involved in the reduction of organic nitrogen compounds and ammonia production. Defects in the生物素酶抗体 gene encoding biotinidase are the cause of biotinidase deficiency, which is characterized by skin rash, ataxia, seizures, hearing loss, hypotonia and optic atrophy. These symptoms are due to the individual’s inability to reutilize biotin and can, therefore, typically be treated with the addition of free biotin. Function: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. Subcellular Location: Secreted. DISEASE: Defects in BTD are the cause of biotinidase deficiency (BTD deficiency) [MIM:253260]; also called late-onset multiple carboxylase deficiency. BTD生物素酶抗体 deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur. Similarity: Belongs to the CN hydrolase family. BTD/VNN subfamily. Contains 1 CN hydrolase domain. Gene ID: 686 Database links: Entrez Gene: 686 Human Omim: 609019 Human SwissProt: P43251 Human Unigene: 517830 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
生物素酶抗体产品应用: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 神经生物学 信号转导
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid