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Details on Person UniProt:Q96HY7 DHTKD1
| Class:Id | ReferenceGeneProduct:226982 |
|---|---|
| _chainChangeLog | transit peptide:1- added on Fri February 6 2015;chain:-919 added on Fri February 6 2015 |
| _displayName | UniProt:Q96HY7 DHTKD1 |
| _timestamp | 2025-05-21 21:11:21 |
| chain | transit peptide:1- chain:-919 |
| checksum | CD1E437F919324B6 |
| comment | FUNCTION 2-oxoadipate dehydrogenase (E1a) component of the 2-oxoadipate dehydrogenase complex (OADHC) (PubMed:29191460, PubMed:29752936, PubMed:32303640, PubMed:32633484, PubMed:32695416). Participates in the first step, rate limiting for the overall conversion of 2-oxoadipate (alpha-ketoadipate) to glutaryl-CoA and CO(2) catalyzed by the whole OADHC (PubMed:29191460, PubMed:32695416). Catalyzes the irreversible decarboxylation of 2-oxoadipate via the thiamine diphosphate (ThDP) cofactor and subsequent transfer of the decarboxylated acyl intermediate on an oxidized dihydrolipoyl group that is covalently amidated to the E2 enzyme (dihydrolipoyllysine-residue succinyltransferase or DLST) (Probable) (PubMed:29752936, PubMed:32303640, PubMed:32633484). Can catalyze the decarboxylation of 2-oxoglutarate in vitro, but at a much lower rate than 2-oxoadipate (PubMed:29191460, PubMed:29752936, PubMed:32633484, PubMed:32695416). Responsible for the last step of L-lysine, L-hydroxylysine and L-tryptophan catabolism with the common product being 2-oxoadipate (Probable).CATALYTIC ACTIVITY N(6)-[(R)-lipoyl]-L-lysyl-[protein] + 2-oxoadipate + H(+) = N(6)-[(R)-S(8)-glutaryldihydrolipoyl]-L-lysyl-[protein] + CO2COFACTOR Amino-acid degradation.SUBUNIT The 2-oxoadipate dehydrogenase complex is composed of OADH (2-oxoadipate dehydrogenase; E1a), DLST (dihydrolipoamide succinyltransferase; E2) and DLD (dihydrolipoamide dehydrogenase; E3). E1a functional unit is a dimer. Interacts with DLST (PubMed:32695416).INTERACTION The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS The mitochondrial 2-oxoglutarate and 2-oxoadipate dehydrogenase complexes (OGDHC and OADHC, respectively) share their E2 (DLST) and E3 (dihydrolipoyl dehydrogenase or DLD) components, but the E1 component is specific to each complex (E1o and E1a, respectively).SIMILARITY Belongs to the alpha-ketoglutarate dehydrogenase family.SEQUENCE CAUTION Extended N-terminus.SEQUENCE CAUTION Chimeric cDNA. C-terminal exons are derived from the neighboring gene. |
| created | [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53 |
| description | recommendedName: 2-oxoadipate dehydrogenase complex component E1 shortName evidence="16 19"E1a shortName: OADC-E1 shortName: OADH-E1 ecNumber evidence="12 14 21 25"1.2.4.- alternativeName: 2-oxoadipate dehydrogenase, mitochondrial alternativeName: Alpha-ketoadipate dehydrogenase shortName: Alpha-KADH-E1 alternativeName: Dehydrogenase E1 and transketolase domain-containing protein 1 alternativeName: Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial |
| geneName | DHTKD1 KIAA1630 |
| identifier | Q96HY7 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Charcot-Marie-Tooth disease Disease variant Glycolysis Mitochondrion Neurodegeneration Neuropathy Oxidoreductase Proteomics identification Reference proteome Thiamine pyrophosphate Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 |
| name | DHTKD1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8962379] ENSEMBL:ENSG00000181192 DHTKD1 [Homo sapiens] |
| secondaryIdentifier | DHTK1_HUMAN Q68CU5 Q9BUM8 Q9HCE2 |
| sequenceLength | 919 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:6792669] DHTKD1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792672] lipo-K-DHTKD1 [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ModifiedResidue:6792671] N6-lipoyl-L-lysine at 188 [ModifiedResidue:6792673] N6-lipoyl-L-lysine at 183 [ModifiedResidue:6792674] N6-lipoyl-L-lysine at 818 [ModifiedResidue:6792675] N6-lipoyl-L-lysine at 800 |
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No pathways have been reviewed or authored by UniProt:Q96HY7 DHTKD1 (226982)
