Gene-dose effect on carnitine transport activity in embryonic fibroblasts of JVS mice as a model of human carnitine transporter deficiency.

@article{Hashimoto1998GenedoseEO,
  title={Gene-dose effect on carnitine transport activity in embryonic fibroblasts of JVS mice as a model of human carnitine transporter deficiency.},
  author={Naoto Hashimoto and Fumiaki Suzuki and Ikumi Tamai and Hiroko Nikaido and Masamichi Kuwajima and Jun-ichiro Hayakawa and Akira Tsuji},
  journal={Biochemical pharmacology},
  year={1998},
  volume={55 10},
  pages={
          1729-32
        }
}
Recently, the marked decline in renal carnitine reabsorption has been thought to account fotr the systemic carnitine deficiency in juvenile visceral steatosis (JVS) mice. We have conducted a kinetic analysis using embryonic fibroblasts derived from normal, heterozygous, and homozygous jvs mice and found that the high-affinity carnitine transporter (Km = 5.5 microM), which shows Na+ and temperature dependency and stereospecificity, is defective in homozygous jvs mice. Moreover, a gene dose… CONTINUE READING
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