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DHTKD1 Q96HY7

2-己二酸脱氢酶复合物组分E1 (E1a) (OADC-E1) (OADH-E1) (EC 1.2.4.-) (2-己二酸脱氢酶, 线粒体) (α-己二酸脱氢酶) (Alpha-KADH-E1) (含脱氢酶E1和转酮醇酶结构域蛋白1) (推测的2-氧戊二酸脱氢酶E1组分DHKTD1, 线粒体)

蛋白质信息 (UniProt)

2-己二酸脱氢酶复合物组分E1 (E1a) (OADC-E1) (OADH-E1) (EC 1.2.4.-) (2-己二酸脱氢酶, 线粒体) (α-己二酸脱氢酶) (Alpha-KADH-E1) (含脱氢酶E1和转酮醇酶结构域蛋白1) (推测的2-氧戊二酸脱氢酶E1组分DHKTD1, 线粒体)

Q96HY7

功能描述

2-oxoadipate dehydrogenase complex (OADHC)的2-oxoadipate dehydrogenase (E1a)组分 (PubMed:29191460, PubMed:29752936, PubMed:32303640, PubMed:32633484, PubMed:32695416)。参与由整个OADHC催化的2-oxoadipate (alpha-ketoadipate)转化为glutaryl-CoA和CO(2)的第一步反应,该步骤是整个反应的限速步骤 (PubMed:29191460, PubMed:32695416)。通过thiamine diphosphate (ThDP)辅因子催化2-oxoadipate的不可逆脱羧,随后将脱羧的酰基中间体转移到共价酰胺化连接于E2 enzyme (dihydrolipoyllysine-residue succinyltransferase或DLST)的氧化型二氢硫辛酰基团上 (Probable) (PubMed:29752936, PubMed:32303640, PubMed:32633484)。在体外能催化2-oxoglutarate的脱羧,但速率远低于2-oxoadipate (PubMed:29191460, PubMed:29752936, PubMed:32633484, PubMed:32695416)。负责L-lysine、L-hydroxylysine和L-tryptophan分解代谢的最后一步,共同产物为2-oxoadipate (Probable)。 {ECO:0000269|PubMed:29191460, ECO:0000269|PubMed:29752936, ECO:0000269|PubMed:32303640, ECO:0000269|PubMed:32633484, ECO:0000269|PubMed:32695416, ECO:0000305|PubMed:29191460, ECO:0000305|PubMed:29752936, ECO:0000305|PubMed:32303640, ECO:0000305|PubMed:32633484, ECO:0000305|PubMed:32695416}。

亚细胞定位

Mitochondrion

关键词

3D-structure Charcot-Marie-Tooth disease Disease variant Glycolysis Mitochondrion Neurodegeneration Neuropathy Oxidoreductase Proteomics identification Reference proteome