{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:15:42Z","timestamp":1787494542210,"version":"build-2736575974"},"reference-count":50,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2012,10,10]],"date-time":"2012-10-10T00:00:00Z","timestamp":1349827200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Department of Paediatrics, The Hospital for Sick Children, Toronto"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2012,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    <jats:italic>Introduction:<\/jats:italic>\n                    Muscle and cardiac metabolism are dependent on the oxidation of fats and glucose for adenosine triphosphate production, for which\n                    <jats:sc>L<\/jats:sc>\n                    \u2010carnitine is an essential cofactor.\n                    <jats:italic>Methods:<\/jats:italic>\n                    We measured muscle carnitine concentrations in skeletal muscles, diaphragm, and ventricles of C57BL\/10ScSn\u2010DMD\n                    <jats:italic>mdx<\/jats:italic>\n                    \/J mice (\n                    <jats:italic>n<\/jats:italic>\n                    = 10) and compared them with wild\u2010type C57BL\/6J (\n                    <jats:italic>n<\/jats:italic>\n                    = 3), C57BL\/10 (\n                    <jats:italic>n<\/jats:italic>\n                    = 10), and C3H (\n                    <jats:italic>n<\/jats:italic>\n                    = 12) mice. Citrate synthase (CS) activity was measured in quadriceps\/gluteals and ventricles of\n                    <jats:italic>mdx<\/jats:italic>\n                    and wild\u2010type mice.\n                    <jats:italic>Results:<\/jats:italic>\n                    We found significantly lower tissue carnitine in quadriceps\/gluteus (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.05) and ventricle (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.05), but not diaphragm of\n                    <jats:italic>mdx<\/jats:italic>\n                    mice, when compared with controls. CS activity was increased in\n                    <jats:italic>mdx<\/jats:italic>\n                    quadriceps\/gluteus (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.03) and ventricle (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.02). This suggests compensatory mitochondrial biogenesis.\n                    <jats:italic>Conclusions:<\/jats:italic>\n                    Decreased tissue carnitine has implications for reduced fatty acid and glucose oxidation in\n                    <jats:italic>mdx<\/jats:italic>\n                    quadriceps\/gluteus and ventricle. The\n                    <jats:italic>mdx<\/jats:italic>\n                    mouse may be a useful model for studying the role of muscle carnitine deficiency in DMD bioenergetic insufficiency and providing a targeted and timed rationale for\n                    <jats:sc>L<\/jats:sc>\n                    \u2010carnitine therapy. Muscle Nerve 46: 767\u2013772, 2012\n                  <\/jats:p>","DOI":"10.1002\/mus.23368","type":"journal-article","created":{"date-parts":[[2012,3,4]],"date-time":"2012-03-04T02:35:14Z","timestamp":1330828514000},"page":"767-772","source":"Crossref","is-referenced-by-count":4,"title":["The\n                    <i>mdx<\/i>\n                    mouse as a model for carnitine deficiency in the pathogenesis of duchenne muscular dystrophy"],"prefix":"10.1002","volume":"46","author":[{"given":"Zarazuela","family":"Zolkipli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia","family":"Mai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne\u2010Marie","family":"Lamhonwah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingrid","family":"Tein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,10,10]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(87)90579-4"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.122.4.809"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.1192"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005545325254"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01738755"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(00)00201-7"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(01)00205-X"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1096\/fj.01-0030fje"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/352536a0"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880151008"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1006868130002"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1098\/rspb.1993.0030"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj3040649"},{"key":"e_1_2_6_15_2","first-page":"12","article-title":"Carnitine deficiency, mitochondrial dysfunction and the heart. 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