Forearm 3-methylhistidine efflux in myotonic dystrophy.
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.
Rifai
Z
Z
Department of Neurology, University of Rochester, NY 14642.
Kingston
W J
WJ
McCraith
B
B
Moxley
R T
RT
D000328:Adult; D015992:Body Mass Index; D002851:Chromatography, High Pressure Liquid; D005542:Forearm / Q000098:blood supply*; D006801:Humans; D008297:Male; D008762:Methylhistidines / Q000032:analysis*; D008875:Middle Aged; D009133:Muscular Atrophy / Q000503:physiopathology*; D009210:Myofibrils / Q000378:metabolism; D009223:Myotonic Dystrophy / Q000503:physiopathology*; D012016:Reference Values; D012039:Regional Blood Flow
D008762:Methylhistidines; C028118:3-methylhistidine