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 Metabolism and Citrate cycle (TCA cycle). Gene Ontology (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
Dihydrolipoamide succinyltransferase (E2) component of the 2-oxoglutarate dehydrogenase complex (By similarity). The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2) (By similarity). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion (PubMed:29211711). A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A (PubMed:29211711).