Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency

We have reported the clinical and biochemical findings in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. This paper is the first report about cardiomyopathy in jvs mice. Adult jvs mice (at the age of 2 3 months) show cardiac hypertrophy which is caused by enlargement of t...

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Bibliographic Details
Published in:FEBS letters 1993-07, Vol.326 (1), p.267-271
Main Authors: Horiuchi, Masahisa, Yoshida, Hiroki, Kobayashi, Keiko, Kuriwaki, Kazumi, Yoshimine, Kosei, Tomomura, Mineko, Koizumi, Tsutomu, Nikaido, Hiroko, Hayakawa, Junichiro, Kuwajima, Masamichi, Saheki, Takeyori
Format: Article
Language:English
Subjects:
Animal model
Animals
Biological and medical sciences
Cardiac hypertrophy
Cardiology. Vascular system
Cardiomegaly - etiology
Cardiomegaly - pathology
Cardiomegaly - prevention & control
Carnitine - administration & dosage
Carnitine - deficiency
Carnitine - therapeutic use
Cell Nucleus - pathology
Cytoplasm - pathology
DNA - metabolism
Fatty Liver - complications
Fatty Liver - pathology
FBP, fructose 1,6-bisphosphate
Female
GOT, glutamic oxaloacetic transaminase
Heart
Homozygote
Juvenile visceral steatosis mice
jvs, juvenile visceral steatosis
Male
Medical sciences
Mice
Mice, Inbred C3H
Mice, Mutant Strains
Myocarditis. Cardiomyopathies
Myocardium - pathology
NCP, non-collagen protein
Organ Size
SDH, succinate dehydrogenase
Systemic carnitine deficiency
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Summary:We have reported the clinical and biochemical findings in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. This paper is the first report about cardiomyopathy in jvs mice. Adult jvs mice (at the age of 2 3 months) show cardiac hypertrophy which is caused by enlargement of the cardiac muscle cell associated with increases of 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 important role in the development of the cardiac hypertrophy. The discovery of cardiac hypertrophy in carnitine-deficient jvs mice will lead to clarification of the pathophysiology of cardiomyopathy in systemic carnitine deficiency in human beings.
ISSN:0014-5793
1873-3468
DOI:10.1016/0014-5793(93)81805-A