| 619859 | Dlat | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity; acetyltransferase activity (ortholog); identical protein binding (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA; pyruvate catabolic process (ortholog); tricarboxylic acid cycle (ortholog); PARTICI PATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Sleep Deprivation; Chemical and Drug Induced Liver Injury (ortholog); genetic disease (ortholog); FOUND IN mitochondrial matrix; pyruvate dehydrogenase complex; mitochondrion (ortholog); INTERACTS WITH (R)-adrenaline; 17alpha-ethynylestradiol; 2,2,2-tetramine | 8 | 59875537 | 59900947 | Rat | 215 | symbol , PhenoGen | gene, protein-coding, PROVISIONAL [RefSeq] |
| 735273 | DLAT | dihydrolipoamide S-acetyltransferase | This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed b y the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009] | 11 | 112025408 | 112064404 | Human | 305 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 735274 | Dlat | dihydrolipoamide S-acetyltransferase | Enables acetyltransferase activity. Involved in pyruvate catabolic process and pyruvate decarboxylation to acetyl-CoA. Located in mitochondrion and myelin sheath. Is active in mitochondrial matrix. Is expressed in several structures, including alimentary system; genitourinary system; heart; nervous system; and respiratory system. Human ortholog(s) of this gene implicated in pyruvate decarboxylase deficiency. Orthologous to human DLAT (dihydrolipoamide S-acetyltransferase). [provided by Alliance of Genome Resources, Apr 2025] | 9 | 50545933 | 50571080 | Mouse | 228 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8911676 | Dlat | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits acetyltransferase activity (ortholog); dihydrolipoyllysine-residue acetyltransferase activity (ortholog); identical protein binding (ortholog); INVOLVED IN pyruvate catabolic process (ortholog); pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Liver Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrion (ortholog); pyruvate dehydrogenase complex (ortholog) | NW_004955412 | 13718091 | 13746220 | Chinchilla | 39 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11963190 | DLAT | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Live r Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN pyruvate dehydrogenase complex (ortholog) | 11 | 106910857 | 106950851 | Bonobo | 54 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12388517 | DLAT | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Live r Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN pyruvate dehydrogenase complex (ortholog) | 5 | 21062833 | 21094235 | Dog | 52 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12514388 | Dlat | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Live r Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN pyruvate dehydrogenase complex (ortholog) | NW_004936612 | 2395452 | 2437592 | Squirrel | 54 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626100370 | Dlat | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits acetyltransferase activity (ortholog); dihydrolipoyllysine-residue acetyltransferase activity (ortholog); identical protein binding (ortholog); INVOLVED IN pyruvate catabolic process (ortholog); pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Liver Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrion (ortholog); pyruvate dehydrogenase complex (ortholog) | | | | Black Rat | 38 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18900394 | Dlat | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Live r Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN pyruvate dehydrogenase complex (ortholog) | | | | Naked Mole-Rat | 53 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18542962 | DLAT | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); identical protein binding (ortholog); INVOLVED IN pyruvate catabolic process (ortholog); pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria p athway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Liver Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN mitochondrion (ortholog); pyruvate dehydrogenase complex (ortholog) | | | | Green Monkey | 51 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155231612 | dlat | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 13962030 | DLAT | dihydrolipoamide S-acetyltransferase | ENCODES a protein that exhibits dihydrolipoyllysine-residue acetyltransferase activity (ortholog); INVOLVED IN pyruvate decarboxylation to acetyl-CoA (ortholog); PARTICIPATES IN citric acid cycle pathway; fumaric aciduria pathway; Leigh disease pathway; ASSOCIATED WITH Chemical and Drug Induced Live r Injury (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN pyruvate dehydrogenase complex (ortholog) | | | | Pig | 53 | symbol | gene, protein-coding, PROVISIONAL [RefSeq] |
| 14380282 | dlat-1 | | | | | | Roundworm | | symbol | gene, null |
| 14382945 | dlat-2 | | | | | | Roundworm | | symbol | gene, null |
| 155241244 | dlat.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 1583609 | Dlat-ps1 | dihydrolipoamide S-acetyltransferase, pseudogene 1 | INTERACTS WITH bisphenol A | 1 | 35059647 | 35061656 | Rat | 1 | symbol , PhenoGen | gene, pseudo, MODEL [RefSeq] |
