Griggs R C, Halliday D, Kingston W, Moxley R T
Ann Neurol. 1986 Nov;20(5):590-6. doi: 10.1002/ana.410200506.
Muscle wasting in myotonic dystrophy may result from decreased muscle anabolic processes rather than from increased catabolism. Male patients with myotonic dystrophy often have low levels of circulating androgens, and androgen administration has been shown to increase their muscle mass. We have studied the effect of testosterone enanthate administration (3 mg/kg weekly for 3 months) on muscle and whole body protein synthesis in 6 male patients with myotonic dystrophy. Muscle protein synthesis was estimated from the rate of isotope incorporation into muscle protein obtained by quadriceps muscle biopsy during a primed continuous infusion of L-[1-13C]leucine. Testosterone administration resulted in a significant increase in muscle protein synthesis in all patients. Whole body protein synthesis did not increase, indicating that protein synthesis in other tissues may have declined. Muscle ribonucleic acid content rose significantly in response to testosterone administration, suggesting that testosterone initiated its effect by hormone receptor interaction with muscle nuclei.
强直性肌营养不良中的肌肉萎缩可能是由于肌肉合成代谢过程减少，而非分解代谢增加所致。患有强直性肌营养不良的男性患者通常循环雄激素水平较低，而雄激素给药已被证明可增加其肌肉质量。我们研究了庚酸睾酮给药（每周3mg/kg，共3个月）对6例男性强直性肌营养不良患者肌肉和全身蛋白质合成的影响。在持续静脉输注L-[1-13C]亮氨酸的同时，通过股四头肌活检获得肌肉蛋白质中同位素掺入率，以此估算肌肉蛋白质合成。给予睾酮后，所有患者的肌肉蛋白质合成均显著增加。全身蛋白质合成并未增加，这表明其他组织中的蛋白质合成可能有所下降。给予睾酮后，肌肉核糖核酸含量显著上升，这表明睾酮通过与肌肉细胞核的激素受体相互作用发挥其效应。
