The genetic deletion of desmin from <i>mdx^4cv</i> mice reduces body mass and survival.

<p><b>A</b>) Desmin expression was significantly increased in the <i>mdx^4cv</i> skeletal muscles. Bars represent the mean +/− S.D. densitometry of desmin expression from n = 7 wild-type and n = 6 <i>mdx^4cv</i> gastrocnemius muscles. <b>B</b>) Photograph of representative wild-type and dko mice. Note that the dko mice develop a mild kyphosis at 11 weeks of age. <b>C</b>) Confirmation of genotype by immunostaining of frozen gastrocnemius muscle sections with antibodies to dystrophin and desmin. Scale bar = 50 µm <b>D</b>) Mean +/− S.D. body mass of wild-type (n = 5), <i>mdx^4cv</i> (n = 4), desmin^−/− (n = 5) and dko mice (n = 9). <b>E</b>) Kaplan-Meyer survival analyses demonstrating the significantly shortened lifespan of dko mice (n = 13) compared with desmin^−/− mice (n = 16). *<i>P</i><0.05, **<i>P</i><0.01 compared with wild-type. ^@@@P<0.001 compared to desmin^−/−.