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货号： bs-18959R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-18959R
- 英文名称
- MMAB
- 中文名称
- 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/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 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
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.
- 分 子 量
- 24kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human MMAB:101-200/250
- 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.
- PubMed
- PubMed
- 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.
SWISS:
Q96EY8
Gene ID:
236625
Database links:Entrez Gene: 326625Human
Entrez Gene: 77697Mouse
Omim: 607568Human
SwissProt: Q96EY8Human
SwissProt: Q9D273Mouse
Unigene: 12106Human
Unigene: 105182Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.