生物素酶抗体

生物素酶抗体英文名称  Anti-BTD/Biotinidase/Sp8 

中文名称  生物素酶抗体 

别    名  Biotinase; Biotinidase; Btd; BTD_HUMAN; EC 3.5.1.12. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg 

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep   

产品类型  一抗    

研究领域  细胞生物 神经生物学 信号转导  

蛋白分子量  predicted molecular weight: 57kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human Biotinidase 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

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.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

生物素酶抗体产品介绍 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 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 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.

Database links : UniProtKB/Swiss-Prot: P43251.2