PDHX Proteins and Enzymes

The PDHX gene encodes component X of the pyruvate dehydrogenase (PDH) complex. For a detailed description of the pyruvate dehydrogenase complex, see MIM 300502. The mammalian PDH complex differs from that in E. coli and from the other mammalian alpha-keto acid dehydrogenases by the presence of a 53-kD protein called protein X. Component X binds to the E3 (MIM 238331) component of the PDH complex (Robinson et al., 1990; Aral et al., 1997).[supplied by OMIM]