Dihydrolipoyl transacetylase
Dihydrolipoyl transacetylase | ||
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PDB 1Y8O | ||
Properties of human protein | ||
Mass / length primary structure | 561 amino acids | |
Cofactor | 2 lipoate | |
Identifier | ||
Gene name | DLAT | |
External IDs | ||
Enzyme classification | ||
EC, category | 2.3.1.12 , Transferase | |
Response type | Transfer of an acetyl residue | |
Substrate | Acetyl-CoA + Enzyme- N (6) - (Dihydrolipoyl) lysine | |
Products | CoA + enzyme- N (6) - ( S -acetyldihydrolipoyl) lysine | |
Occurrence | ||
Parent taxon | Creature |
The dihydrolipoyl transacetylase ( E2 , correctly dihydrolipoyllysine residue acetyltransferase , also dihydrolipoamide-S-acetyltransferase , PDHE2 ) is the enzyme that functions as subunit E2 of the pyruvate dehydrogenase complex (PDH) for the maintenance of the glycolysis in all living things and the citric acid cycle . Within the PDH E2 has the task of transferring of the acetyl from acetyl-CoA to the bonded PDH lipoic acid - cofactor and back. In humans, PDHE2 is the autoallergen in primarily biliary cirrhosis , an autoimmune disease . Mutations in the DLAT - gene are the cause of certain forms of pyruvate dehydrogenase deficiency .
In primary biliary cirrhosis, E2 is presumably first modified by methyl octinolate occurring in the vicinity , whereupon it becomes such a strong antigen that antibodies against normal E2 are also formed during apoptosis.
Individual evidence
- ↑ UniProt P10515
- ↑ Amano K, Leung PS, Rieger R, et al. : Chemical xenobiotics and mitochondrial autoantigens in primary biliary cirrhosis: identification of antibodies against a common environmental, cosmetic, and food additive, 2-octynoic acid . In: J. Immunol. . 174, No. 9, May 2005, pp. 5874-83. PMID 15845458 .
- ↑ English JS, Rycroft RJ: Allergic contact dermatitis from methyl heptine and methyl octine carbonates . In: Contact Dermatitis . 18, No. 3, March 1988, pp. 174-5. PMID 2966714 .
Web links
- Citric acid cycle with pyruvate dehydrogenase position and color representations of molecules. Archived from the original on February 21, 2014 ; accessed on January 17, 2016 .
- OrphaNet: Pyruvate dehydrogenase deficiency