硫辛酰胺脱氢酶抗体
规格：1mg/1ml
英文名： DLDD/Lipoamide Dehydrogenase
别名： Dehydrogenase complex, E3 component; Diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase; Dihydrolipoyl dehydrogenase mitochondrial; dld; DLDD; DLDH; DLDH_HUMAN; E3; E3 branched ch
分子量： 50kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human DLDD/Lip
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Sheep, Xenopus laevis
细胞定位：
硫辛酰胺脱氢酶抗体产品介绍：background: This gene encodes a member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014] Function: Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction. 硫辛酰胺脱氢酶抗体Subcellular Location: Mitochondrion matrix. Post-translational modifications: Tyrosine phosphorylated. DISEASE: Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis. Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids 硫辛酰胺脱氢酶抗体of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation. Similarity: Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. Gene ID: 1738 Database links: Entrez Gene: 1738 Human Entrez Gene: 403978 Dog Entrez Gene: 13382 Mouse Entrez Gene: 397129 Pig Entrez Gene: 298942 Rat Omim: 238331 Human SwissProt: P49819 Dog SwissProt: P09622 Human SwissProt: O08749 Mouse SwissProt: P09623 Pig SwissProt: Q6P6R2 Rat Unigene: 131711 Human Unigene: 3131 Mouse Unigene: 86962 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
硫辛酰胺脱氢酶抗体产品应用：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：肿瘤 细胞生物 信号转导 新陈代谢 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid