| 產(chǎn)品編號 | bs-14345R |
| 英文名稱 | Rabbit Anti-DMGDH antibody |
| 中文名稱 | 二甲基甘氨酸脫氫酶抗體 |
| 別 名 | Dimethylglycine dehydrogenase; Dimethylglycine dehydrogenase, mitochondrial; Dmgdh; M2GD_HUMAN; ME2GLYDH; mitochondrial. |
| 研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 新陳代謝 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 91kDa |
| 細(xì)胞定位 | 細(xì)胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DMGDH: 101-200/866 |
| 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 | 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 an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013] Subcellular Location: Mitochondrion. DISEASE: Defects in DMGDH are the cause of DMGDH deficiency (DMGDHD) [MIM:605850]. DMGDHD is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. Biochemically it is characterized by an increase of N,N-dimethylglycine (DMG) in serum and urine. Similarity: Belongs to the GcvT family. SWISS: Q9UI17 Gene ID: 29958 Database links: Entrez Gene: 29958 Human Entrez Gene: 100724353 Guinea pig Entrez Gene: 100073267 Horse Entrez Gene: 74129 Mouse Entrez Gene: 100340207 Rabbit Omim: 605849 Human SwissProt: Q9UI17 Human SwissProt: Q9DBT9 Mouse Unigene: 655653 Human Unigene: 21789 Mouse Unigene: 3646 Rat |
