| Primary Identifier | IPR045257 | Type | Family |
| Short Name | E2/Pdx1 |
| description | In prokaryotes and eukaryotes, pyruvate dehydrogenase complex (PDC) contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3) []. PDC catalyses the overall conversion of pyruvate to acetyl-CoA and CO2. In eukaryotes, PDC is located in the mitochondrial matrix [, ]. This entry represents agroup of E3-binding proteins, including dihydrolipoamide acetyltransferase (E2) and pyruvate dehydrogenase protein X component (known as Pdx1). Pdx1, found only in eukaryotes, plays a structural role in the complex by binding and positioning dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide acetyltransferase (E2) core []. Mutations in the Pdx1 gene has been linked to human disease [, ]. Mutations in the E2 gene cause pyruvate dehydrogenase deficiency []. |
