Differential susceptibility of muscles to myotonia and force impairment in a mouse model of myotonic dystrophy.

Abstract:

INTRODUCTION:Myotonic dystrophy, or dystrophia myotonica (DM), is characterized by prominent muscle wasting and weakness as well as delayed muscle relaxation resulting from persistent electrical discharges. METHODS:We hypothesized heterogeneity among muscles in degree of weakness and myotonia in an expanded [(CUG)(250)] repeats transgenic (HSA(LR)) mouse DM model. Muscle contraction was compared among diaphragm, extensor digitorum longus (EDL), and soleus muscles. RESULTS:Myotonia was found only in EDL, as manifested by longer late-relaxation time and elevated myotonic index. EDL, but not the other two muscles, had impaired force over a wide range of stimulation frequencies. During fatigue-inducing stimulation, DM EDL muscle force per cross-sectional area was significantly impaired during 25-Hz stimulation, whereas there were no differences in fatigue response for DM diaphragm or soleus. CONCLUSION:In an expanded repeats model of DM the EDL is more susceptible to myotonia and force impairment than muscles with lower proportions of fast-twitch fibers.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Moyer M,Berger DS,Ladd AN,Van Lunteren E

doi

10.1002/mus.21988

subject

Has Abstract

pub_date

2011-06-01 00:00:00

pages

818-27

issue

6

eissn

0148-639X

issn

1097-4598

journal_volume

43

pub_type

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