2-氧戊二酸脱氢酶复合物组分E1 (E1o) (HsOGDH) (OGDC-E1) (OGDH-E1) (EC 1.2.4.2) (2-氧戊二酸脱氢酶, 线粒体) (α-酮戊二酸脱氢酶) (Alpha-KGDH-E1) (焦磷酸硫胺素 (ThDP)依赖性2-氧戊二酸脱氢酶)
2-氧戊二酸脱氢酶复合物组分E1 (E1o) (HsOGDH) (OGDC-E1) (OGDH-E1) (EC 1.2.4.2) (2-氧戊二酸脱氢酶, 线粒体) (α-酮戊二酸脱氢酶) (Alpha-KGDH-E1) (焦磷酸硫胺素 (ThDP)依赖性2-氧戊二酸脱氢酶)
Q02218功能描述
2-oxoglutarate dehydrogenase complex (OGDHC)的2-oxoglutarate dehydrogenase (E1o)组分 (PubMed:24495017, PubMed:25210035, PubMed:28435050)。参与整个OGDHC催化的2-oxoglutarate转化为succinyl-CoA和CO(2)的第一步反应，该步骤是整个反应过程的限速步骤 (PubMed:24495017, PubMed:25210035, PubMed:28435050)。通过thiamine diphosphate (ThDP)辅因子催化2-oxoglutarate (alpha-ketoglutarate)的不可逆脱羧，随后将脱羧的酰基中间体转移至共价酰胺化连接在E2 enzyme (dihydrolipoyllysine-residue succinyltransferase or DLST)上的氧化型二氢硫辛酰基团 (PubMed:24495017, PubMed:25210035, PubMed:28435050, PubMed:35272141)。在Krebs (citric acid)循环中起关键作用，该循环是包括碳水化合物、脂肪酸和氨基酸在内的燃料分子氧化的共同途径 (PubMed:25210035)。在体外能催化2-oxoadipate的脱羧，但速率远低于2-oxoglutarate (PubMed:28435050)。也能将2-keto-4-hydroxyglutarate (KHG)和CoA转化为malyl-CoA (By similarity)。主要在线粒体中具有活性 (PubMed:29211711)。一部分2-oxoglutarate dehydrogenase complex也定位于细胞核，是组蛋白lysine succinylation所必需的：与染色质上的KAT2A结合，为histone succinyltransferase KAT2A提供succinyl-CoA (PubMed:29211711)。 {ECO:0000250|UniProtKB:K9IVI1, ECO:0000269|PubMed:24495017, ECO:0000269|PubMed:25210035, ECO:0000269|PubMed:28435050, ECO:0000269|PubMed:29211711, ECO:0000303|PubMed:25210035}。
亚细胞定位
Mitochondrion
关键词