| 產(chǎn)品編號 | bsm-63329R |
| 英文名稱 | ACADM Recombinant Rabbit mAb |
| 中文名稱 | ?；o酶A脫氫酶中鏈重組兔單抗 |
| 別 名 | mitochondrial antibody; ACAD 1; ACAD1; Acyl coenzyme A dehydrogenase; Acyl coenzyme A dehydrogenase C 4 to C 12 straight chain; MCAD; MCADH; Medium chain acyl CoA dehydrogenase; Medium chain fatty acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase mitochondrial; FLJ18227; FLJ93013; FLJ99884; ACADM_HUMAN. |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號 | 5C9 |
| 交叉反應(yīng) | Human |
| 產(chǎn)品應(yīng)用 | IHC-P=1:50-200, IHC-F=1:50-200, IF=1:50-200, Flow-Cyt=1μg/Test
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 47kDa |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ACADM |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] Function: This enzyme is specific for acyl chain lengths of 4 to 16. Subunit: Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF. Subcellular Location: Mitochondrion matrix. DISEASE: Acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:201450]: An inborn error of mitochondrial fatty acid beta-oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the acyl-CoA dehydrogenase family. SWISS: P11310 Gene ID: 34 Database links: ?；o酶，是一種特殊的酰基鏈長度為4-16的脫氫酶，它催化脂肪酸β氧化的起始步驟。利用電子轉(zhuǎn)移黃蛋白（ETF）作為電子受體，通過ETF-泛醌氧化還原酶（ETF脫氫酶）將電子轉(zhuǎn)移到線粒體呼吸鏈。 |
