Reactome | PDH E1 decarboxylates PYR, transferring acetyl to DLAT

PDH E1 decarboxylates PYR, transferring acetyl to DLAT

Stable Identifier

R-HSA-9861734

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

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PDH E1 decarboxylates PYR, transferring acetyl to DLAT

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The E1 component of the pyruvate dehydrogenase complex (PDC, PDHC) catalyzes the decarboxylation of pyruvate (PYR) with subsequent transfer of acetyl to the E2 subunit (DLAT). E1 is a complex consisting of a heterotetramer of two PDHA1 or PDHA2 (testis-specific) subunits with two PDHB subunits. Cofactors are two thiamin diphosphate molecules, as well as two Mg2+ ions and two K+ ions (Ciszak et al., 2003). PDH complex deficiencies can be caused by defects in PDHA1 (PDHAD, MIM:312170), PDHA2 (SPGF70, MIM:619828), and PDHB (PDHBD, MIM:614111). For a review of PDH complex deficiency, see Prasad et al., 2011.

Literature References

PubMed ID	Title	Journal	Year
12651851	Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenase Patel, MS, Korotchkina, LG, Ciszak, EM, Dominiak, PM, Sidhu, S	J Biol Chem	2003
21908116	Pyruvate dehydrogenase deficiency and epilepsy Rupar, T, Prasad, AN, Prasad, C	Brain Dev	2011