CATH Classification

Domain Context

CATH Clusters

Superfamily 3.40.50.970
Functional Family Pyruvate dehydrogenase E1 component subunit beta

Enzyme Information

1.2.4.1
Pyruvate dehydrogenase (acetyl-transferring).
based on mapping to UniProt P11177
Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).
-!- It is a component (in multiple copies) of the multienzyme pyruvate dehydrogenase complex in which it is bound to a core of molecules of EC 2.3.1.12, which also binds multiple copies of EC 1.8.1.4. -!- It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.12.

UniProtKB Entries (1)

P11177
ODPB_HUMAN
Homo sapiens
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

PDB Structure

PDB 6CFO
External Links
Method X-RAY DIFFRACTION
Organism
Primary Citation
Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the alpha V138M variant of human pyruvate dehydrogenase.
Whitley, M.J., Arjunan, P., Nemeria, N.S., Korotchkina, L.G., Park, Y.H., Patel, M.S., Jordan, F., Furey, W.
J. Biol. Chem.