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Immunogen sequence: SGQDVERGT FSHRHHVLHD QNVDKRTCIP MNHLWPNQAP YTVCNSSLSE YGVLGFELGF AMASPNALVL WEAQFGDFHN TAQCIIDQFI CPGQAKWVRQ NGIVLLLPHG MEGMGPEHSS ARPERFLQMC NDDPDVLPDL KEANFDINQL YDCNWVVVNC STPGNFFHVL RRQILLPFRK PLIIFTPKSL LRHPEARSSF DEMLPGTHFQ RVIPEDGPAA QNPENVKRLL FCTGKVYYDL TRERKARDMV GQVAITRIEQ LSPFPFDLLL KEVQKYPNAE LAWCQEEHKN QGYYDYVKPR LRTTISRAKP VWYAGRDPAA APATGNKKTH LTELQRLLDT AFDLDVFKNF S (674-1023 aa encoded by BC004964)
This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitocondrial matrix and uses thiamine pyrophosphate as a cofactor. A congential deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia.
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Protein Aliases: 2-oxoglutarate dehydrogenase complex component E1; 2-oxoglutarate dehydrogenase E1 component, mitochondrial; 2-oxoglutarate dehydrogenase, mitochondrial; Alpha-ketoglutarate dehydrogenase; Alpha-KGDH-E1; E1o; OGDC-E1; oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide); oxoglutarate decarboxylase; oxoglutarate dehydrogenase (lipoamide); oxoglutarate dehydrogenase (succinyl-transferring); testicular tissue protein Li 131; Thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase
Gene Aliases: AKGDH; E1k; OGDC; OGDH; Ogdhl
Molecular Function: dehydrogenase