Dihydrolipoyl Transacetylase (DLAT)

DLTA; PDC-E2; PBC; M2 antigen complex 70 kDa subunit; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Pyruvate dehydrogenase complex E2

SPN	W116
HGNC	2896
MGI	2385311
PubMed	7487063
RGD	619859
UniProt	P10515
Wiki	DLAT

Dihydrolipoyl transacetylase is the 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 by 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.

Reference

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Submitted (OCT-2006) to the PDB data bank

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