Kuwajima M, Lu K, Sei M, Ono A, Hayashi M, Ishiguro K, Ozaki K, Hotta K, Okita K, Murakami T, Miyagawa J, Narama I, Nikaido H, Hayakawa J, Nakajima H, Namba M, Hanafusa T, Matsuzawa Y, Shima K
Department of Laboratory Medicine, School of Medicine, University of Tokushima, Japan.
J Mol Cell Cardiol. 1998 Apr;30(4):773-81. doi: 10.1006/jmcc.1998.0641.
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.
幼年内脏脂肪变性（JVS）小鼠表现出遗传性全身肉碱缺乏，并会发展为心脏肥大。本研究的目的是阐明JVS小鼠心脏肥大的特征。在4周龄和8周龄时，IVS小鼠心脏中的总肉碱含量约为对照小鼠心脏的10%。在2周龄时，JVS小鼠的心脏重量/体重比高于对照小鼠，且这种比例差异随年龄增加。在2周龄和8周龄时，JVS小鼠的左右心室壁和室间隔壁面积均大于对照小鼠。JVS小鼠心室壁和室间隔的心肌细胞直径长于对照小鼠。电子显微镜观察显示，JVS小鼠心肌细胞中的线粒体百分比为66%，对照小鼠为37%。JVS小鼠的脂质分数百分比比对照小鼠高六倍。JVS小鼠心脏中腺嘌呤核苷酸的总含量约为对照小鼠心脏的60%。在4周龄和8周龄时，JVS小鼠心脏的腺苷酸能荷分别为对照小鼠心脏的63%和45%。总体而言，在该动物模型中观察到的心脏增大可归因于心室和室间隔中心肌细胞直径的成比例增加，同时伴有线粒体增加。此外，这种细胞生长与ATP、ADP水平以及腺苷酸能荷的降低有关。
