翻訳と辞書 Words near each other ・ Glutamyl endopeptidase ・ Glutamyl endopeptidase II ・ Glutamyl-tRNA reductase ・ Glutaraldehyde ・ Glutarate-semialdehyde dehydrogenase ・ Glutarate—CoA ligase ・ Glutaredoxin ・ Glutaredoxin 2 (bacterial) ・ Glutaric acid ・ Glutaric acidemia type 2 ・ Glutaric aciduria type 1 ・ Glutarimide ・ Glutaronitrile ・ Glutaryl-7-aminocephalosporanic-acid acylase ・ Glutaryl-CoA ・ Glutaryl-CoA dehydrogenase ・ Glutaryl-CoA dehydrogenase (non-decarboxylating) ・ Glutathione ・ Glutathione amide reductase ・ Glutathione amide-dependent peroxidase ・ Glutathione dehydrogenase (ascorbate) ・ Glutathione disulfide ・ Glutathione gamma-glutamylcysteinyltransferase ・ Glutathione hydrolase ・ Glutathione oxidase ・ Glutathione peroxidase ・ Glutathione reductase ・ Glutathione S-transferase ・ Glutathione S-transferase A1 ・ Glutathione S-transferase Mu 1 Dictionary Lists mini英和辞書 mini和英辞書 Webster 1913 Latin-English FOLDOC Wikipedia English ウィキペディア

翻訳と辞書　辞書検索 [ 開発暫定版 ] スポンサード リンク Glutaryl-CoA dehydrogenase ： ウィキペディア英語版 Glutaryl-CoA dehydrogenase Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD). It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants.〔(【引用サイトリンク】Homo sapiens (human) )">url=http://www.ncbi.nlm.nih.gov/gene/2639 )〕 ==Structure== GCDH is a tetramer with tetrahedral symmetry, which allows it to be seen as a dimer of dimers. Its structure is very similar to other ACDs but the overall polypeptide fold of the GCDH is made up of three domains: an alpha-helical bundle amino-terminal domain, a beta-sheet domain in the middle, and another alpha-helical domain at the carboxyl terminus. The flavin adenine dinucleotide (FAD) is located at the junction between the middle beta-strand and the carboxyl terminal alpha-helix domain of one subunit and the carboxyl-terminal domain of the neighboring subunit. The most distinct difference between GCDH and other ACDs in terms of structure is the carboxyl and amino-terminal regions of the monomer and in the loop between beta-strands 4 and 5 because it is only made up of four residues, whereas other ACDs have much more. The substrate-binding pocket is filled with a string of three water molecules, which gets displaced when the substrate binds to the enzyme. The binding pocket is also smaller than some of the other ACD binding pockets because it is responsible for the chain-length specificity of GCDH for alternate substrates. The GCDH gene is mapped onto 19p13.2 and has an exon count of 15. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Glutaryl-CoA dehydrogenase」の詳細全文を読む スポンサード リンク 翻訳と辞書 : 翻訳のためのインターネットリソース Copyright(C) kotoba.ne.jp 1997-2016. All Rights Reserved.