DIFFERENTIAL EXPRESSION OF MUSCULAR-DYSTROPHY IN DIAPHRAGM VERSUS HINDLIMB MUSCLES OF MDX MICE

Contractile properties of diaphragm (DIA) from mdx and control mice were compared with those of hindlimb muscles [soleus (SOL) and extensor digitorum longus (EDL)] in vitro. Mice ranged in age from 2 weeks to 1.5 years. Muscles were directly stimulated and properties measured were: contraction time, half-relaxation time, active tension per unit area, fatigue index, and maximal velocity of shortening (V(max)). Active tension decreased significantly with age in mdx DIA but not in control DIA. SOL and EDL active tensions were less in mdx than control over the whole age range and did not decrease with age. V(max) was decreased in mdx DIA, but not in mdx SOL or EDL. These results demonstrate that DIA is more affected by muscular dystrophy than hindlimb muscles. Since many Duchenne patients exhibit respiratory distress, this differential expression of dystrophy in diaphragm, as compared to limb muscles, may have important clinical implications.