Aliases for DLST Gene
External Ids for DLST Gene
Previous HGNC Symbols for DLST Gene
Previous GeneCards Identifiers for DLST Gene
This gene encodes a mitochondrial protein that belongs to the 2-oxoacid dehydrogenase family. This protein is one of the three components (the E2 component) of the 2-oxoglutarate dehydrogenase complex that catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Oct 2011]
GeneCards Summary for DLST Gene
DLST (Dihydrolipoamide S-Succinyltransferase) is a Protein Coding gene. Diseases associated with DLST include Alpha-Ketoglutarate Dehydrogenase Deficiency and Wernicke-Korsakoff Syndrome. Among its related pathways are Histidine, lysine, phenylalanine, tyrosine, proline and tryptophan catabolism and Pyruvate metabolism and Citric Acid (TCA) cycle. GO annotations related to this gene include transferase activity and dihydrolipoyllysine-residue succinyltransferase activity. An important paralog of this gene is DBT.
UniProtKB/Swiss-Prot for DLST Gene
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).