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Truncated dystrophins reduce muscle stiffness in the extensor digitorum longus muscle of mdx mice

Posted: February 17, 2013 at 2:52 am

Muscle stiffness is a major clinical feature in Duchenne muscular dystrophy (DMD). DMD is the most common lethal inherited muscle-wasting disease in boys, and it is caused by the lack of the dystrophin protein. We recently showed that the extensor digitorum longus (EDL) muscle of mdx mice (a DMD mouse model) exhibits disease-associated muscle stiffness. Truncated micro- and mini-dystrophins are the leading candidates for DMD gene therapy. Unfortunately, it has never been clear whether these truncated genes can mitigate muscle stiffness. To address this question, we examined the passive properties of the EDL muscle in transgenic mdx mice that expressed a representative mini- or micro-gene (H2-R15, R2-15/R18-23/C, or R4-23/C). The passive properties were measured at the ages of 6 and 20 mo a...

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