CARDIAC-HYPERTROPHY IN JUVENILE VISCERAL STEATOSIS (JVS) MICE WITH SYSTEMIC CARNITINE DEFICIENCY

We have reported the clinical and biochemical findings in juvenile vis ceral steatosis (jvs) mice with systemic carnitine deficiency. This pa per is the first report about cardiomyopathy in jvs mice. Adult jvs mi ce (at the age of 2-3 months) show cardiac hypertrophy which is caused by enlargement of the cardiac muscle cell associated with increases o f non-collagen protein and DNA content. Carnitine administration (2 mg /head, twice a day, from 1 month of age) significantly suppresses the cardiac hypertrophy, showing that carnitine deficiency plays an import ant role in the development of the cardiac hypertrophy. The discovery of cardiac hypertrophy in carnitine-deficient jvs mice will lead to cl arification of the pathophysiology of cardiomyopathy in systemic carni tine deficiency in human beings.