Steatohepatitis is characterized by abnormal liver lipid metabolism and altered mitochondrial function. Carnitine is an essential cofactor for mitochondrial beta oxidation of fatty acids. This study examined the effect of carnitine supplementation on liver function and peripheral blood mitochondria copy number in patients with non alcoholic fatty liver disease (NAFLD). Forty-five NAFLD patients were assigned to a carnitine group or a control group. Liver function tests and analysis of peripheral blood mitochondrial DNA were performed before and following three months of treatment. Carnitine treatment was associated with reduction of ALT, AST, and total bilirubin. In comparison, there was no change in AST, ALT, and total bilirubin associated with treatment in the control group. Peripheral mitochondrial DNA copy number was significantly increased from 158.8+/- 69.5 copies to 241.6+/-180.6 copies (p=0.025) in the carnitine group, but not in the control group. Carnitine improved liver function and mitochondrial numbers in NAFLD. (Korean J Gastroenterol. 2010 Jun;55(6):384-9.) PMID: 20571306.