肌生成抑制蛋白的缺失减轻了mdx小鼠的肌肉萎缩严重程度。
Loss of myostatin attenuates severity of muscular dystrophy in mdx mice.
作者信息
Wagner Kathryn R, McPherron Alexandra C, Winik Nicole, Lee Se-Jin
机构信息
Department of Neurology, The Johns Hopkins University, 725 N. Wolfe Street, Baltimore, MD 21205, USA.
出版信息
Ann Neurol. 2002 Dec;52(6):832-6. doi: 10.1002/ana.10385.
Myostatin, a transforming growth factor-beta family member, is a negative regulator of skeletal muscle growth. To explore the therapeutic potential of targeting myostatin in settings of muscle degeneration, we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mdx mice lacking myostatin were stronger and more muscular than their mdx counterparts. Diaphragm muscle showed less fibrosis and fatty remodeling, suggesting improved muscle regeneration.
肌肉生长抑制素是转化生长因子-β家族成员，是骨骼肌生长的负调节因子。为了探索在肌肉退化情况下靶向肌肉生长抑制素的治疗潜力，我们将肌肉生长抑制素基因敲除的突变小鼠与mdx小鼠（杜氏和贝克型肌营养不良症模型）进行杂交。缺乏肌肉生长抑制素的mdx小鼠比其mdx同窝小鼠更强壮且肌肉更发达。膈肌显示出更少的纤维化和脂肪重塑，表明肌肉再生得到改善。
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