Kuwajima M, Lu K, Harashima H, Ono A, Sato I, Mizuno A, Murakami T, Nakajima H, Miyagawa J, Namba M, Hanafusa T, Hayakawa J, Matsuzawa Y, Shima K
Department of Laboratory Medicine, School of Medicine, Tokushima University, Japan.
Biochem Biophys Res Commun. 1996 Jun 14;223(2):283-7. doi: 10.1006/bbrc.1996.0885.
Juvenile visceral steatosis (JVS) mice are associated with systemic carnitine deficiency (Kuwajima, et al., 1991). In order to investigate the cause of this deficiency, we compared fibroblast carnitine transport activities in normal mice and JVS mice. The kinetic analysis showed that in formal fibroblasts, the Km and Vmax values for saturable uptake was 15.6 microM and 2.56 pmol/min/mg protein, respectively. In JVS fibroblasts, however, saturable uptake was not observed. There was no great difference in the linear component of uptake between normal and JVS fibroblasts. At the physiological concentration (50 microM) of carnitine, the fibroblast carnitine transport activity in JVS mice was decreased to 18% of that in normal mice. Thus there is hardly any carnitine transport activity in the fibroblasts of JVS mice, indicating that the JVS mouse can be regarded as an animal model of primary carnitine deficiency.
青少年内脏脂肪变性（JVS）小鼠与全身性肉碱缺乏有关（Kuwajima等人，1991年）。为了研究这种缺乏的原因，我们比较了正常小鼠和JVS小鼠成纤维细胞的肉碱转运活性。动力学分析表明，在正常成纤维细胞中，可饱和摄取的Km和Vmax值分别为15.6微摩尔和2.56皮摩尔/分钟/毫克蛋白质。然而，在JVS成纤维细胞中，未观察到可饱和摄取。正常和JVS成纤维细胞摄取的线性成分没有很大差异。在肉碱的生理浓度（50微摩尔）下，JVS小鼠成纤维细胞的肉碱转运活性降至正常小鼠的18%。因此，JVS小鼠的成纤维细胞几乎没有肉碱转运活性，这表明JVS小鼠可被视为原发性肉碱缺乏的动物模型。
