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500ug
| 产品编号 | bs-13312P |
| 英文名称 | GCDH Antibody Blocking Peptide |
| 中文名称 | 戊二酰辅酶A脱氢酶封闭多肽 |
| 英文别名 | ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781. |
| 纯化方法 | HPLC |
| 研究领域 | Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers Signal Transduction > Metabolism > Amino Acids Signal Transduction > Metabolism > Mitochondrial Tags & Cell Markers > Subcellular Markers > Organelles > Mitochondria |
| 亚基 | Homotetramer. |
| 亚细胞定位 | Mitochondrion matrix. |
| 组织特异性 | Isoform 1 and isoform 2 are expressed in fibroblasts and liver. |
| 相似性 | Belongs to the acyl-CoA dehydrogenase family. |
| 功能 | 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. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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 such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. |
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文献和实验Dual-Color ELISPOT Assay for the Simultaneous Detection of IL-2 and/or IFN- Secreting T Cells
, activate AEC buffer using H2 O2 (see Step 25). Anti-CD3 monoclonal antibody (mAb) (Fisher Scientific) Blocking buffer for ELISPOT assay CEF peptide pool (available from the NIH AIDS Research and Reagent Program and other commercial
0.3% hydrogen peroxide (prepare immediately before use) Blocking buffer (see recipe ) HCV‐specific primary antibody (Ab), e.g
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