|
|||||
|
|
Primary defect of juvenile visceral steatosis (jvs) mouse with systemic carnitine deficiency is probably in renal carnitine transport system |
|
| Authors: | Masahisa Horiuchi Keiko Kobayashi Seiji Yamaguchi Nobuo Shimizu Tsutomu Koizumi Hiroko Nikaido Jun-ichiro Hayakawa Masamichi Kuwajima Takeyori Saheki |
| Institution: | 1. Department of Biochemistry, Faculty of Medicine, Kagoshima University, Kagoshima, Japan;2. Department of Pediatrics, Gifu University School of Medicine, Gifu, Japan;3. Intitute for Experimental Animals, Fukui Medical School, Fukui, Japan;4. Institute for Experimental Animals, School of Medicine, Kanazawa University, Kanazawa, Japan;5. Department of Internal Medicine, Osaka University Medical School, Osaka, Japan |
| Abstract: | We investigated the reabsorptional system for carnitine in the kidney to elucidate the mechanism of carnitine deficiency in juvenile visceral steatosis (jvs) mice. Jvs mice had a higher rate of carnitine excretion at 10 days after birth than the controls, in spite of having no pathological acylcarnitine in the urine. In an experiment to assay the uptake of carnitine using kidney slices, homozygous mutants showed significantly lower rates of Na-dependent carnitine uptake than controls. Heterozygous mice showed values of transport activity intermediate between homozygous mutants and homozygous controls. Scatchard plots (transport activity versus transport activity/carnitine concentration) revealed that the homozygous mutants had a defect in the hihg affinity site (Km = 58 μM) in the Na-dependent carnitine transport system in the kidney. These results indicate that the primary defect of jvs mice is most probably related to the system for reabsorption of carnitine in the kidney. |
| Keywords: | Systemic carnittine deficiency Reabsorption of carnitine Na-dependent transport system (Animal model) juvenile visceral steatosis FAB/MS fast atom bombardment/mass spectrometry GC/MS gas chromatography/ mass spectrometry |
| 本文献已被 ScienceDirect 等数据库收录！ | |