Identification of the up- and down-regulated genes in the heart of juvenile visceral steatosis mice.

Juvenile visceral steatosis (JVS) mice, novel animal models of systemic carnitine deficiency, exhibit a remarkably increased number of mitochondria in their cardiac myocytes. To date, however, there has been no reported investigation of the molecular mechanism of this increased number of mitochondria. Here, we analyzed the gene expression profile from the hearts of JVS and control mice by Affymetrix GeneChip analysis representing 34323 genes.

Functional disorders of the oxidative phosphorylation system in the heart mitochondria of mice with juvenile visceral steatosis.

Mice with juvenile visceral steatosis (JVS) develop remarkable cardiac hypertrophy and exhibit an increased number of mitochondria in their heart. However, the biochemical characteristics and physiological functions of these mitochondria cardiac are little known. Here we show that the respiratory activities at state 3 with glutamate plus malate or succinate in the heart mitochondria of JVS mice were greatly decreased to 47% or 77%, respectively, compared with those of control mice.