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Exercise increases utrophin protein expression in the mdx mouse model of Duchenne muscular dystrophy
Author(s) -
Gordon Bradley S.,
Lowe Dawn A.,
Kostek Matthew C.
Publication year - 2014
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24151
Subject(s) - duchenne muscular dystrophy , utrophin , mdx mouse , muscular dystrophy , dystrophin , protein expression , medicine , physical medicine and rehabilitation , biology , genetics , gene
: 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