{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T11:39:10Z","timestamp":1779104350787,"version":"3.51.4"},"reference-count":33,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2014,3,19]],"date-time":"2014-03-19T00:00:00Z","timestamp":1395187200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001718","name":"Netherlands Organization for International Cooperation in Higher Education","doi-asserted-by":"publisher","award":["016.086.336"],"award-info":[{"award-number":["016.086.336"]}],"id":[{"id":"10.13039\/501100001718","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","award":["200320006"],"award-info":[{"award-number":["200320006"]}],"id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2014,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Very long\u2010chain acyl\u2010CoA dehydrogenase (VLCAD) deficiency is an inherited disorder of mitochondrial long\u2010chain fatty acid \u03b2\u2010oxidation (FAO). Patients with VLCAD deficiency may present with hypoglycemia, hepatomegaly, cardiomyopathy, and myopathy. Although several mouse models have been developed to aid in the study of the pathogenesis of long\u2010chain FAO defects, the muscular phenotype is underexposed. To address the muscular phenotype, we used a newly developed mouse model on a mixed genetic background with a more severe defect in FAO (\n                    <jats:italic>\n                      LCAD\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    ;\n                    <jats:italic>VLCAD<\/jats:italic>\n                    <jats:sup>+\/\u2013<\/jats:sup>\n                    ) in addition to a validated mouse model (\n                    <jats:italic>LCAD<\/jats:italic>\n                    <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <jats:italic>\n                      ; VLCAD\n                      <jats:sup>+\/+<\/jats:sup>\n                    <\/jats:italic>\n                    ) and compared them with wild\u2010type (WT) mice. We found that both mouse models show a 20% reduction in energy expenditure (EE) and a 3\u2010fold decrease in locomotor activity in the unfed state. In addition, we found a 1.7\u00b0C drop in body temperature in unfed\n                    <jats:italic>LCAD<\/jats:italic>\n                    <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <jats:italic>\n                      ; VLCAD\n                      <jats:sup>+\/+<\/jats:sup>\n                    <\/jats:italic>\n                    mice compared with WT body temperature. We conclude that food withdrawal\u2010induced inactivity, hypothermia, and reduction in EE are novel phenotypes associated with FAO deficiency in mice. Unexpectedly, inactivity was not explained by rhabdomyolysis, but rather reflected the overall reduced capacity of these mice to generate heat. We suggest that mice are partly protected against the negative consequence of an FAO defect.\u2014Diekman, E. F., van Weeghel, M., Wanders, R. J. A., Visser, G., Houten, S. M. Food withdrawal lowers energy expenditure and induces inactivity in long\u2010chain fatty acid oxidation\u2013deficient mouse models.\n                    <jats:italic>FASEB J<\/jats:italic>\n                    . 28, 2891\u20132900 (2014).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.14-250241","type":"journal-article","created":{"date-parts":[[2014,3,19]],"date-time":"2014-03-19T23:00:11Z","timestamp":1395270011000},"page":"2891-2900","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Food withdrawal lowers energy expenditure and induces inactivity in long\u2010chain fatty acid oxidation\u2013deficient mouse models"],"prefix":"10.1096","volume":"28","author":[{"given":"Eugene F.","family":"Diekman","sequence":"first","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital University Medical Center (UMC) Utrecht the Netherlands"},{"name":"Department of Clinical Chemistry Laboratory Genetic Metabolic Diseases University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel","family":"van Weeghel","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Laboratory Genetic Metabolic Diseases University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald J. A.","family":"Wanders","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Laboratory Genetic Metabolic Diseases University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Pediatrics Emma Children's Hospital Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gepke","family":"Visser","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital University Medical Center (UMC) Utrecht the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sander M.","family":"Houten","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Laboratory Genetic Metabolic Diseases University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Pediatrics Emma Children's Hospital Academic Medical Center University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Genetics and Genomic Sciences Icahn Institute for Genomics and Multiscale Biology Icahn School of Medicine at Mount Sinai New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,3,19]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.100.22.2248"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.64.082201.154705"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10500"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005504223140"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI0215311"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/clpt.2010.55"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc0806334"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000118"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9061-2"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.19.2069"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.0000088786.19197.E4"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00811.2005"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.26.15592"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2009.05.006"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M800221-JLR200"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt382"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.111.963751"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvt212"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005587617745"},{"key":"e_1_2_7_21_1","volume-title":"Methods of Enzymatic Analysis","author":"Bergmeyer H. 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