title: Characteristics of cardiac hypertrophy in the juvenile visceral steatosis mouse with systemic carnitine deficiency.
abstract: The juvenile visceral steatosis (JVS) mouse exhibits hereditary systemic carnitine deficiency and develops cardiac hypertrophy. The aim of this study was to clarify the characteristics of cardiac hypertrophy in the JVS mouse. Total carnitine content in IVS mouse heart was about 10% of that of control mouse heart at 4 and 8 weeks of age. The heart weight/body weight ratio was bigger in JVS mice than that in control mice at 2 weeks of age, and this difference in ratio increased with age. The wall areas of both ventricles and septum in JVS mice were larger than those of the control mice at 2 and 8 weeks. The myocyte diameter in both ventricular walls and septum in JVS mice was longer than that of the control mice. On electron microscopy, the percent of mitochondria in the myocyte was 66% in JVS mice, and 37% in control mice. The percent of lipid fraction in JVS mice was six-fold higher than that in control mice. Total content of adenine nucleotides in JVS mouse heart was about 60% of that in control mouse heart. Adenylate energy charge in JVS mouse heart was 63 and 45% of that in the control mouse heart at 4 and 8 weeks, respectively. Overall, the cardiac enlargement observed in this animal model could be accounted for by a proportional increase in the myocyte diameter in the ventricles and septum, accompanied by an increase in mitochondria. Furthermore, this cellular growth is associated with decreases in the levels of ATP and ADP, and adenylate energy charge.
authors.affiliation: Department of Laboratory Medicine, School of Medicine, University of Tokushima, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kuwajima
authors.forename: K
authors.initials: K
authors.lastname: Lu
authors.forename: M
authors.initials: M
authors.lastname: Sei
authors.forename: A
authors.initials: A
authors.lastname: Ono
authors.forename: M
authors.initials: M
authors.lastname: Hayashi
authors.forename: K
authors.initials: K
authors.lastname: Ishiguro
authors.forename: K
authors.initials: K
authors.lastname: Ozaki
authors.forename: K
authors.initials: K
authors.lastname: Hotta
authors.forename: K
authors.initials: K
authors.lastname: Okita
authors.forename: T
authors.initials: T
authors.lastname: Murakami
authors.forename: J
authors.initials: J
authors.lastname: Miyagawa
authors.forename: I
authors.initials: I
authors.lastname: Narama
authors.forename: H
authors.initials: H
authors.lastname: Nikaido
authors.forename: J
authors.initials: J
authors.lastname: Hayakawa
authors.forename: H
authors.initials: H
authors.lastname: Nakajima
authors.forename: M
authors.initials: M
authors.lastname: Namba
authors.forename: T
authors.initials: T
authors.lastname: Hanafusa
authors.forename: Y
authors.initials: Y
authors.lastname: Matsuzawa
authors.forename: K
authors.initials: K
authors.lastname: Shima
mesh_terms: D000244:Adenosine Diphosphate / Q000378:metabolism; D000249:Adenosine Monophosphate / Q000378:metabolism; D000255:Adenosine Triphosphate / Q000378:metabolism; D000818:Animals; D006332:Cardiomegaly / Q000378:metabolism* / Q000473:pathology; D002331:Carnitine / Q000172:deficiency* / Q000378:metabolism; D051379:Mice; D008809:Mice, Inbred C3H; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology
chemical_list: D000249:Adenosine Monophosphate; D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate; D002331:Carnitine