顿2贬骋顿贬蛋白抗体
规格：1尘驳/1尘濒
英文名： D2HGDH
别名： D-2-hydroxyglutarate dehydrogenase; D-2-hydroxyglutarate dehydrogenase, mitochondrial; D2HGD; D2HDH_HUMAN; D2hgdh.
分子量： 56kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human D2HGDH
交叉反应：Human, Mouse, Rat, Rabbit,
细胞定位：
顿2贬骋顿贬蛋白抗体产物介绍：background: This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. [provided by RefSeq, Jul 2008] Function: Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate. Disease: Defects in D2HGDH are the cause of D-2-hydroxyglutaric aciduria type 1 (D2HGA1) [MIM:600721]. D2HGA1 is a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia,顿2贬骋顿贬蛋白抗体 and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine. Similarity: Belongs to the FAD-binding oxidoreductase/transferase type 顿2贬骋顿贬蛋白抗体4 family. Contains 1 FAD-binding PCMH-type domain. Gene ID: 728294 Database links: Entrez Gene: 728294 Human Omim: 609186 Human SwissProt: Q8N465 Human Unigene: 516813 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
顿2贬骋顿贬蛋白抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid