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Muscle Nerve ; 63(2): 239-249, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33125736

RESUMO

INTRODUCTION: We conducted an open-label study to examine the effects of the flavonoid (-)-epicatechin in seven ambulatory adult patients with Becker muscular dystrophy (BMD). METHODS: Seven participants received (-)-epicatechin 50 mg twice per day for 8 weeks. Pre- and postprocedures included biceps brachii biopsy to assess muscle structure and growth-relevant endpoints by western blotting, mitochondria volume measurement, and cristae abundance by electron microscopy, graded exercise testing, and muscle strength and function tests. RESULTS: Western blotting showed significantly increased levels of enzymes modulating cellular bioenergetics (liver kinase B1 and 5'-adenosine monophosphate-activated protein kinase). Peroxisome proliferator-activated receptor gamma coactivator-1alpha, a transcriptional coactivator of genes involved in mitochondrial biogenesis and cristae-associated mitofilin levels, increased as did cristae abundance. Muscle and plasma follistatin increased significantly while myostatin decreased. Markers of skeletal muscle regeneration myogenin, myogenic regulatory factor-5, myoblast determination protein 1, myocyte enhancer factor-2, and structure-associated proteins, including dysferlin, utrophin, and intracellular creatine kinase, also increased. Exercise testing demonstrated decreased heart rate, maximal oxygen consumption per kilogram, and plasma lactate levels at defined workloads. Tissue saturation index improved in resting and postexercise states. DISCUSSION: (-)-Epicatechin, an exercise mimetic, appears to have short-term positive effects on tissue biomarkers indicative of mitochondrial biogenesis and muscle regeneration, and produced improvements in graded exercise testing parameters in patients with BMD.


Assuntos
Catequina/uso terapêutico , Músculo Esquelético/metabolismo , Distrofia Muscular de Duchenne/tratamento farmacológico , Adulto , Biópsia , Western Blotting , Creatina Quinase/metabolismo , Disferlina/metabolismo , Teste de Esforço , Folistatina/metabolismo , Frequência Cardíaca , Humanos , Ácido Láctico/sangue , Fatores de Transcrição MEF2/metabolismo , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade , Mitocôndrias/ultraestrutura , Proteínas Mitocondriais/metabolismo , Tamanho Mitocondrial , Proteínas Musculares/metabolismo , Força Muscular , Músculo Esquelético/fisiopatologia , Músculo Esquelético/ultraestrutura , Distrofia Muscular de Duchenne/metabolismo , Distrofia Muscular de Duchenne/patologia , Distrofia Muscular de Duchenne/fisiopatologia , Proteína MyoD/metabolismo , Fator Regulador Miogênico 5/metabolismo , Miogenina/metabolismo , Miostatina/metabolismo , Biogênese de Organelas , Consumo de Oxigênio , Coativador 1-alfa do Receptor gama Ativado por Proliferador de Peroxissomo/metabolismo , Regeneração , Utrofina/metabolismo
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