It was reported that serum guanidinoacetic acid (GAA) levels are elevated in acute hapatic failure. To explore the mechanism of this GAA level change, the activities of kidney glycine amidinotransferase (GAT) and liver GAA-methyltransferase (GAA-MT) were measured in experimental rat models of acute hepatic failure. GAA is synthesized by the former enzyme and catabolized by the latter enzyme. In the early stage, kidney GAT activity was increased (P<0.1) but the serum GAA level remained normal. In the late stage, liver GAA-MT activity was decreased (P<0.01) and serum GAA level was increased. From this result, it is concluded that GAA synthesis is increased in acute hepatic failure and that serum GAA level is increased when liver is severely damaged.