{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T10:23:33Z","timestamp":1779099813623,"version":"3.51.4"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":5814,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1999,10]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Juvenile visceral steatosis (JVS) mice, which show systemic <jats:italic toggle=\"yes\">l<\/jats:italic>-carnitine deficiency, may be an animal model of Reye's syndrome because of its phenotype of fat deposition and mitochondrial abnormalities in the liver. In this study, we compared the characteristics of the L-carnitine transport in isolated hepatocytes from wild-type and JVS mice. The uptake of <jats:italic toggle=\"yes\">l<\/jats:italic>-carnitine by wild-type hepatocytes was saturable and the Eadie-Hofstee plot showed 2 distinct components. The apparent Michaelis constant (<jats:italic toggle=\"yes\">K<\/jats:italic>\n                                 <jats:sub>m<\/jats:sub>) and the maximum transport rate (<jats:italic toggle=\"yes\">V<\/jats:italic>\n                                 <jats:sub>max<\/jats:sub>) were 4.6 \u03bcmol\/L and 59.5 pmol\/15 min\/10<jats:sup>6<\/jats:sup> cells, respectively, for the high-affinity component, and 404 \u03bcmol\/L and 713 pmol\/15 min\/10<jats:sup>6<\/jats:sup> cells, respectively, for the low-affinity component. The high-affinity <jats:italic toggle=\"yes\">l<\/jats:italic>-carnitine uptake occurred via an active carrier-mediated transport mechanism, which is characterized by Na<jats:sup>+<\/jats:sup>-, energy-, and pH-dependency. On the other hand, the high-affinity uptake was absent in JVS hepatocytes, and the values of <jats:italic toggle=\"yes\">K<\/jats:italic>\n                                 <jats:sub>m<\/jats:sub> and <jats:italic toggle=\"yes\">V<\/jats:italic>\n                                 <jats:sub>max<\/jats:sub> for the low-affinity uptake were 475 \u03bcmol\/L and 557 pmol\/15 min\/10<jats:sup>6<\/jats:sup> cells, respectively. The hepatic carnitine transport properties in wild-type hepatocytes were similar to those of high-affinity mouse Octn2-transfected HEK293 cells. This study suggests that Octn2-type carnitine transporter is dysfunctional in hepatocytes of JVS mice.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.510300423","type":"journal-article","created":{"date-parts":[[2004,7,28]],"date-time":"2004-07-28T07:29:13Z","timestamp":1090999753000},"page":"997-1001","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":34,"title":["Loss of wild-type carrier-mediated L-carnitine transport activity in hepatocytes of juvenile visceral steatosis mice"],"prefix":"10.1097","volume":"30","author":[{"given":"Koichi","family":"Yokogawa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayuki","family":"Yonekawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikumi","family":"Tamai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rikiya","family":"Ohashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuaki","family":"Tatsumi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhiko","family":"Higashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaaki","family":"Nomura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noriyoshi","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroko","family":"Nikaido","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun-ichiro","family":"Hayakawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun-ichi","family":"Nezu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asuka","family":"Oku","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miyuki","family":"Shimane","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken-ichi","family":"Miyamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Tsuji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"BIB1","doi-asserted-by":"crossref","first-page":"3628","DOI":"10.1016\/S0021-9258(19)84498-8","volume":"237","author":"Bremer","year":"1962","journal-title":"J Biol Chem"},{"key":"BIB2","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1152\/ajplegacy.1964.206.3.531","volume":"206","author":"Fritz","year":"1964","journal-title":"Am J Physiol"},{"key":"BIB3","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/0887-8994(90)90037-2","volume":"6","author":"Breningstall","year":"1990","journal-title":"Pediatr Neurol"},{"key":"BIB4","first-page":"351","volume":"28","author":"Scholte","year":"1990","journal-title":"J Clin Chem Clin Biochem"},{"key":"BIB5","doi-asserted-by":"crossref","first-page":"1331","DOI":"10.1056\/NEJM198811173192006","volume":"17","author":"Treem","year":"1988","journal-title":"N Engl J Med"},{"key":"BIB6","first-page":"1508","volume-title":"The Metabolic and Molecular Bases of Inherited Diseases","author":"Roe","unstructured":"Mitochondrial fatty acid oxidation disorder. 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