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Different functional alleles for each subtype of cytochrome P450 (CYPs), thiopurine S-methyltransferase (TPMT), glutathione S-transferase (GST) and sulfotransferase (SULT) enzymes, have been identified. In this review, the genetic polymorphisms of drug-metabolizing enzymes in the Japanese population and their effects on metabolic activity are described in detail.