Skeletal muscle catabolism in amyotrophic lateral sclerosis and chronic spinal muscular atrophy

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Abstract
ALS and chronic spinal muscular atrophy are characterized by wasting of skeletal muscle, suggesting accelerated catabolism or reduced synthesis of muscle protein. We studied seven patients with ALS and three with chronic spinal muscular atrophy using 24-hour urinary 3-methylhistidine excretion as a measure of the rate of muscle catabolism and 24-hour urinary creatinine excretion as an index of muscle mass. The ratio of 3-methylhistidine to creatinine excretion was significantly and similarly higher in both groups of patients than in controls (p < 0.0005), implying a state of accelerated skeletal muscle protein catabolism in these diseases.

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