甲基丙二酸尿症B型蛋白抗体
规格：1mg/1ml
英文名： MMAB
别名： aquocob(I)alamin vitamin B12s adenosyltransferase; ATP:cob(I)alamin adenosyltransferase; ATP:corrinoid adenosyltransferase; ATR; c-diamide adenosyltransferase; cblB; Cob; Cob(I)alamin adenosyltransfer
分子量： 24kDa
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 MMAB
交叉反应：Human, Mouse, Rat, Chicken, Pig, Cow, Rabbit, Sheep,
细胞定位：
甲基丙二酸尿症B型蛋白抗体产品介绍：background: This gene encodes a protein that catalyzes the final step in the conversion of vitamin B(12) into adenosylcobalamin (AdoCbl), a vitamin B12-containing coenzyme for methylmalonyl-CoA mutase. Mutations in the gene are the cause of vitamin B12-dependent methylmalonic aciduria linked to the cblB complementation group. Alternatively spliced transcript variants have been found. [provided by RefSeq, Apr 2011] Subcellular Location: Mitochondrion. Tissue Specificity: Expressed in liver and skeletal muscle. DISEASE: Defects in MMAB are the cause of methylmalonic aciduria type cblB (MMAB) [MIM:251110]; also known as methylmalonic aciduria 甲基丙二酸尿症B型蛋白抗体type B or vitamin B12-responsive methylmalonicaciduria of cblB complementation type. MMAB is a disorder of methylmalonate and cobalamin metabolism due to defective synthesis of adenosylcobalamin. Inheritance is autosomal recessive. Similarity:甲基丙二酸尿症B型蛋白抗体 Belongs to the Cob(I)alamin adenosyltransferase family. Gene ID: 236625 Database links: Entrez Gene: 326625 Human Entrez Gene: 77697 Mouse Omim: 607568 Human SwissProt: Q96EY8 Human SwissProt: Q9D273 Mouse Unigene: 12106 Human Unigene: 105182 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
甲基丙二酸尿症B型蛋白抗体产品应用：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 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