In vivo estimation of muscle protein synthesis in myotonic dystrophy

Abstract

The rate of muscle protein synthesis in patients with myotonic dystronic has been studies, and result correlated with total muscle mass. Whole body and skeletal muscle protein synthesis were estimated by stable isotope methodology with a primed, continuous infusion of 1‐[¹³C]leucine with measurement of incorporation of [¹³C] leucine into muscle into muscle protein in biospy samples. Whole body leucine flux, protein synthesis, and protein breakdown were only slightly depressed, but muscle protein synthesis was markedly decreased, in myotonic dystrophy. This depression of muscle protein synthesis in myotonic dystrophy correlates with previous observations of impaired insulin‐induced muscle uptake of amino acids and support the suggestion that muscle wasting in this disease is the consequence of defective anabolism in muscle.