| 產(chǎn)品編號 | bs-18808R-PE-Cy5 |
| 英文名稱 | Rabbit Anti-MUT/Methylmalonyl Coenzyme A mutase/PE-Cy5 Conjugated antibody |
| 中文名稱 | PE-Cy5標(biāo)記的甲基丙二酰異構(gòu)酶抗體 |
| 別 名 | MCM; Methylmalonyl CoA isomerase; Methylmalonyl CoA mutase mitochondrial; Methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase; mitochondrial; Mut; MUTA_HUMAN. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細胞生物 信號轉(zhuǎn)導(dǎo) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, ) |
| 產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MUT/Methylmalonyl Coenzyme A mutase |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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: 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. |
