FITC标记的二甲基甘氨酸脱氢酶抗体
产品名称： FITC标记的二甲基甘氨酸脱氢酶抗体
英文名称： Anti-DMGDH/FITC
产品编号： HZ-14345R-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
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749
- 传真 : 021-60345367
- 邮箱 : www.shzbio.net
Rabbit Anti-DMGDH/FITC Conjugated antibody
FITC标记的二甲基甘氨酸脱氢酶抗体
| 英文名称 | Anti-DMGDH/FITC |
| 中文名称 | FITC标记的二甲基甘氨酸脱氢酶抗体 |
| 别 名 | Dimethylglycine dehydrogenase; Dimethylglycine dehydrogenase, mitochondrial; Dmgdh; M2GD_HUMAN; ME2GLYDH; mitochondrial. |
| 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 神经生物学 信号转导 新陈代谢 线粒体 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, 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 | 91kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DMGDH |
| 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 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. Database links: Entrez Gene: 29958 Human Entrez Gene: 504453 Cow Entrez Gene: 488935 Dog Entrez Gene: 100724353 Guinea pig Entrez Gene: 100073267 Horse Entrez Gene: 74129 Mouse Entrez Gene: 100525563 Pig Entrez Gene: 100340207 Rabbit Entrez Gene: 245961 Rat Omim: 605849 Human SwissProt: Q9UI17 Human SwissProt: Q9DBT9 Mouse SwissProt: Q63342 Rat Unigene: 655653 Human Unigene: 21789 Mouse Unigene: 3646 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application |
该基因编码一种参与胆碱分解代谢的酶，催化二甲基甘氨酸氧化脱甲基形成肌氨酸。该酶被发现为线粒体基质中的单体，并使用黄素腺嘌呤二核苷酸和叶酸作为辅因子。该基因的突变导致二甲基甘氨酸脱氢酶缺乏，其特点是鱼腥味、慢性肌肉疲劳和血清中肌酸激酶的肌型水平升高。选择性剪接导致多个转录变体。[ RefSeq，JUL 2013 ]