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Catabolic situations contribute to the aggravation of symptoms and induce severe metabolic derangement. Treatment for VLCAD\u2010deficiency includes avoidance of fasting and a long\u2010chain fat\u2010restricted and fat\u2010modified diet in which LCFAs are fully or partially replaced by medium\u2010chain triglycerides (MCT). The aim of this work was to investigate the outcome and the effects of long\u2010term treatment in a mouse model of VLCAD\u2010deficiency. The application of a single MCT bolus in a mouse model of VLCAD\u2010deficiency (VLCAD<jats:sup>\u2212\/\u2212<\/jats:sup> mice) immediately prior to exercise protected the muscles from the accumulation of acylcarnitines providing the required energy and it did not affect hepatic lipid metabolism. However, when MCT was applied over the course of a year as a regular part of the diet, female VLCAD<jats:sup>\u2212\/\u2212<\/jats:sup> mice developed a severe clinical phenotype comparable to the human metabolic syndrome. Indeed, they were characterized by massive visceral fat infiltration, hepatosteatosis, disturbed fatty acid composition, hyperlipidemia, and systemic oxidative stress. In contrast, male VLCAD<jats:sup>\u2212\/\u2212<\/jats:sup> mice seemed to be protected and displayed only signs of insulin resistance. Besides the sex\u2010specific response to MCT supplementation with regard to the lipid metabolism, all VLCAD<jats:sup>\u2212\/\u2212<\/jats:sup> mice developed progressive cardiac dysfunction over time which worsened when they were treated with regular MCT resulting in severe dilated cardiomyopathy. While long term use of MCT oil in mice has adverse effects, no such effects have been demonstrated in humans, likely reflecting the differences in long chain fatty acid oxidation between the two species.<\/jats:p>","DOI":"10.1007\/s10545-017-0016-8","type":"journal-article","created":{"date-parts":[[2017,2,28]],"date-time":"2017-02-28T07:36:47Z","timestamp":1488267407000},"page":"317-323","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Very long\u2010chain acyl\u2010CoA dehydrogenase (VLCAD\u2010) deficiency\u2013studies on treatment effects and long\u2010term outcomes in mouse models"],"prefix":"10.1002","volume":"40","author":[{"given":"Sara","family":"Tucci","sequence":"first","affiliation":[{"name":"Department of General Pediatrics, Center for Pediatrics and Adolescent Medicine Medical Centre \u2013 University of Freiburg Mathildenstrasse 1 Freiburg 79106 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