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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Author(s):
Gimenes, A. C. [1] ; Bravo, D. M. [1] ; Napolis, L. M. [1] ; Mello, M. T. [2] ; Oliveira, A. S. B. [3] ; Neder, J. A. [1] ; Nery, L. E. [1]
Total Authors: 7
Affiliation:
[1] Univ Fed Sao Paulo, Setor Funcao Pulmonar & Fisiol Clin Exercicio, Sao Paulo, SP - Brazil
[2] Univ Fed Sao Paulo, Dept Psicobiol, Sao Paulo, SP - Brazil
[3] Univ Fed Sao Paulo, Setor Doencas Neuromusculares, Sao Paulo, SP - Brazil
Total Affiliations: 3
Document type: Journal article
Source: Brazilian Journal of Medical and Biological Research; v. 48, n. 4, p. 354-362, APR 2015.
Web of Science Citations: 3
Abstract

Exercise 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. (AU)