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Ann Neurol. 1993 Nov;34(5):682-6.

Forearm 3-methylhistidine efflux in myotonic dystrophy.

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  • 1Department of Neurology, University of Rochester, NY 14642.


Myotonic dystrophy is associated with progressive muscular atrophy. To define the mechanism of muscle wasting in this disease, we studied myofibrillar proteolysis in vivo in 8 men moderately affected with myotonic dystrophy, and compared the results with those of 10 normal men. Myofibrillar proteolysis was estimated by measuring the 3-methylhistidine arteriovenous difference (A-V) and efflux (Q) across the forearm in the postabsorptive state. Plasma 3-methylhistidine concentrations were determined by high-performance liquid chromatography with postcolumn o-phthalaldehyde derivatization and fluorescence detection. Plasma flow to the forearm muscles (F) was estimated to represent 85% of total forearm plasma flow as determined by the indicator-dilution technique. Forearm 3-methylhistidine efflux was calculated as: Q = F(A-V). Mean muscle mass (24-hour creatinine excretion), lean body mass, and forearm volume were decreased in the patients with myotonic dystrophy, confirming the presence of muscle atrophy. Mean forearm 3-methylhistidine arteriovenous difference and efflux were not significantly different in the two groups. We conclude that myofibrillar protein degradation is not increased in myotonic dystrophy, even when measured in a muscle compartment selectively affected by wasting. Muscle atrophy in myotonic dystrophy is probably the result of defective anabolism rather than accelerated catabolism.

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