FITC标记的甲基丙二酸尿症B型蛋白抗体-抗体-抗体-生物在线
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FITC标记的甲基丙二酸尿症B型蛋白抗体

FITC标记的甲基丙二酸尿症B型蛋白抗体

商家询价

产品名称: FITC标记的甲基丙二酸尿症B型蛋白抗体

英文名称: Anti-MMAB/FITC

产品编号: HZ-18959R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-MMAB/FITC Conjugated antibody 

FITC标记的甲基丙二酸尿症B型蛋白抗体

 

英文名称 Anti-MMAB/FITC
中文名称 FITC标记的甲基丙二酸尿症B型蛋白抗体
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 adenosyltransferase; Cob(I)yrinic acid a; cob(I)yrinic acid a c diamide adenosyltransferase mitochondrial; Methylmalonic aciduria (cobalamin deficiency) cblB type; Methylmalonic aciduria type B protein; MGC20496; mitochondrial; MMAB; MMAB gene; MMAB_HUMAN; OTTHUMP00000240563; OTTHUMP00000240564.  
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  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Pig, Cow, Rabbit, Sheep, 
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 24kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MMAB
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 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 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:
Belongs to the Cob(I)alamin adenosyltransferase family.

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

该基因编码一种蛋白质,该蛋白质催化维生素B(12)转化为腺苷钴胺(AdoCbl),一种含维生素B12的甲基丙二酰辅酶CoA突变酶。基因突变是与CBLB互补组相关的维生素B12依赖性甲基丙二酸尿症的原因。另外,已经发现剪接转录变体。[由RefSeq,APR 2011提供]