戊二酰辅酶础脱氢酶抗体
规格：1尘驳/1尘濒
英文名： GCDH
别名： ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781.
分子量： 43kDa
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 GCDH
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆
戊二酰辅酶础脱氢酶抗体产物介绍：background: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with 戊二酰辅酶础脱氢酶抗体such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Function: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive. Subunit: Homotetramer. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Isoform 1 and isoform 2 are expressed in fibroblasts and liver. DISEASE: Defects in GCDH are the cause 戊二酰辅酶础脱氢酶抗体of glutaric aciduria type 1 (GA1) [MIM:231670]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Similarity: Belongs to the acyl-CoA dehydrogenase family. Database links: UniProtKB/Swiss-Prot: Q92947.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
戊二酰辅酶础脱氢酶抗体产物应用：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 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid