However, the absence of any of the subunits (DLD, Dihydrolipoamide S-Acetyltransferase (DLAT), Pyruvate Dehydrogenase Beta (PDHB), Pyruvate dehydrogenase Component X (PDHX)) results in lactic acidemia [68–70] and DLD deficiency is more severe and causes other complications because it also participates in the branched-chain alpha-keto acid dehydrogenase complex and the alpha-ketoglutarate dehydrogenase complex. Here, PDHB is linked to pyruvate dehydrogenase E3 deficiency.