FITC标记的甲基丙二酸尿症B型蛋白抗体
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]