FITC标记的甲基丙二酰异构酶抗体
产品名称： FITC标记的甲基丙二酰异构酶抗体
英文名称： Anti-MUT/Methylmalonyl Coenzyme A mutase/FITC
产品编号： HZ-18808R-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-MUT/Methylmalonyl Coenzyme A mutase/FITC Conjugated antibody
FITC标记的甲基丙二酰异构酶抗体
| 英文名称 | Anti-MUT/Methylmalonyl Coenzyme A mutase/FITC |
| 中文名称 | FITC标记的甲基丙二酰异构酶抗体 |
| 别 名 | MCM; Methylmalonyl CoA isomerase; Methylmalonyl CoA mutase mitochondrial; Methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase; mitochondrial; Mut; MUTA_HUMAN. |
| 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, |
| 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 | 79kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MUT/Methylmalonyl Coenzyme A mutase |
| 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 the mitochondrial enzyme methylmalonyl Coenzyme A mutase. In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions. Mutations in this gene may lead to various types of methylmalonic aciduria. [provided by RefSeq, Jul 2008] Function: Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM) [MIM:251000]. MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Similarity: Belongs to the methylmalonyl-CoA mutase family. Contains 1 B12-binding domain. Database links: Entrez Gene: 4594 Human Entrez Gene: 422049 Chicken Entrez Gene: 17850 Mouse Entrez Gene: 688517 Rat Entrez Gene: 569581 Zebrafish Omim: 609058 Human SwissProt: P22033 Human SwissProt: P16332 Mouse Unigene: 485527 Human Unigene: 259884 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
本encodes线粒体酶基因的甲基丙二酰CoA变位酶。人类基因的产品，这是一个维生素B12依赖酶的异构化甲基丙二酰辅酶A（catalyzes到琥珀酰辅酶A，而在其他物种的这种酶可能有不同的功能。在这个基因的突变可能会导致不同类型的甲基丙二酸尿症。RefSeq提供的2008年7月[，]