Effect of L-carnitine on exercise performance in patients with mitochondrial myopathy

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dc.contributor.authorGimenes, Ana Cristina Oliveira [UNIFESP]
dc.contributor.authorBravo, Daniela Manzoli [UNIFESP]
dc.contributor.authorNápolis, Lara Maris [UNIFESP]
dc.contributor.authorMello, Marco Tulio de [UNIFESP]
dc.contributor.authorOliveira, Acary Souza Bulle [UNIFESP]
dc.contributor.authorNeder, Jose Alberto [UNIFESP]
dc.contributor.authorNery, Luiz Eduardo [UNIFESP]
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.date.accessioned2015-06-14T13:47:29Z
dc.date.available2015-06-14T13:47:29Z
dc.date.issued2015-01-01
dc.description.abstractExercise intolerance due to impaired oxidative metabolism is a prominent symptom in patients with mitochondrial myopathy (MM), but it is still uncertain whether L-carnitine supplementation is beneficial for patients with MM. The aim of our study was to investigate the effects of L-carnitine on exercise performance in MM. Twelve MM subjects (mean age±SD=35.4±10.8 years) with chronic progressive external ophthalmoplegia (CPEO) were first compared to 10 healthy controls (mean age±SD=29±7.8 years) before they were randomly assigned to receive L-carnitine supplementation (3 g/daily) or placebo in a double-blind crossover design. Clinical status, body composition, respiratory function tests, peripheral muscle strength (isokinetic and isometric torque) and cardiopulmonary exercise tests (incremental to peak exercise and at 70% of maximal), constant work rate (CWR) exercise test, to the limit of tolerance [Tlim]) were assessed after 2 months of L-carnitine/placebo administration. Patients with MM presented with lower mean height, total body weight, fat-free mass, and peripheral muscle strength compared to controls in the pre-test evaluation. After L-carnitine supplementation, the patients with MM significantly improved their Tlim (14±1.9 vs 11±1.4 min) and oxygen consumption ( V ˙ O 2 ) at CWR exercise, both at isotime (1151±115 vs 1049±104 mL/min) and at Tlim (1223±114 vs 1060±108 mL/min). These results indicate that L-carnitine supplementation may improve aerobic capacity and exercise tolerance during high-intensity CWRs in MM patients with CPEO.en
dc.description.affiliationUniversidade Federal de São Paulo (UNIFESP) Setor de Função Pulmonar e Fisiologia Clínica do Exercício
dc.description.affiliationUniversidade Federal de São Paulo (UNIFESP) Departamento de Psicobiologia
dc.description.affiliationUniversidade Federal de São Paulo (UNIFESP) Setor de Doenças Neuromusculares
dc.description.affiliationUnifespUNIFESP, Setor de Função Pulmonar e Fisiologia Clínica do Exercício
dc.description.affiliationUnifespUNIFESP, Depto. de Psicobiologia
dc.description.affiliationUnifespUNIFESP, Setor de Doenças Neuromusculares
dc.description.sourceSciELO
dc.identifierhttp://dx.doi.org/10.1590/1414-431X20143467
dc.identifier.citationBrazilian Journal of Medical and Biological Research. Associação Brasileira de Divulgação Científica, v. 00, n. ahead, p. 000-000, 2015.
dc.identifier.doi10.1590/1414-431X20143467
dc.identifier.fileS0100-879X2015005043467.pdf
dc.identifier.issn0100-879X
dc.identifier.scieloS0100-879X2015005043467
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/8779
dc.language.isoeng
dc.publisherAssociação Brasileira de Divulgação Científica
dc.relation.ispartofBrazilian Journal of Medical and Biological Research
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectExercise toleranceen
dc.subjectMuscle strengthen
dc.subjectMitochondrial myopathyen
dc.subjectL-carnitine supplementationen
dc.titleEffect of L-carnitine on exercise performance in patients with mitochondrial myopathyen
dc.typeinfo:eu-repo/semantics/article
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