Exercise increases utrophin protein expression in the mdx mouse model of Duchenne muscular dystrophy

• Published in: Muscle & nerve Vol. 49; no. 6; pp. 915 - 918
• Main Authors: Gordon, Bradley S., Lowe, Dawn A., Kostek, Matthew C.
• Format: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01.06.2014; Wiley Subscription Services, Inc
• Subjects: Animals; Disease Models, Animal; dystrophin; Female; Gene Expression; genetic disease; Male; Mice; Mice, Inbred mdx; Muscle, Skeletal - metabolism; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscular Dystrophy, Duchenne - metabolism; Muscular Dystrophy, Duchenne - pathology; Muscular Dystrophy, Duchenne - physiopathology; Physical Conditioning, Animal - physiology; RNA, Messenger - metabolism; skeletal muscle; therapy; treatment; Utrophin - metabolism; treatment; dystrophin; skeletal muscle; genetic disease; therapy
• ISSN: 0148-639X; 1097-4598
• DOI: 10.1002/mus.24151

ABSTRACT Introduction: Duchenne muscular dystrophy (DMD) is a lethal genetic disease caused by mutations in the dystrophin gene resulting in chronic muscle damage, muscle wasting, and premature death. Utrophin is a dystrophin protein homologue that increases dystrophic muscle function and reduces pathology. Currently, no treatments that increase utrophin protein expression exist. However, exercise increases utrophin mRNA expression in healthy humans. Therefore, the purpose was to determine whether exercise increases utrophin protein expression in dystrophic muscle. Methods: Utrophin protein was measured in the quadriceps and soleus muscles of mdx mice after 12 weeks of voluntary wheel running exercise or sedentary controls. Muscle pathology was measured in the quadriceps. Results: Exercise increased utrophin protein expression 334 ± 63% in the quadriceps relative to sedentary controls. Exercise increased central nuclei 4 ± 1% but not other measures of pathology. Conclusions: Exercise may be an intervention that increases utrophin expression in patients with DMD. Muscle Nerve 49: 915–918, 2014