Myostatin is a negative regulator of skeletal muscle growth. Myostatin mutations and pharmacological strategies increase muscle mass in vivo, suggesting that myostatin blockade may prove useful in diseases characterized by muscle wasting, such as the muscular dystrophies. We subjected the gamma-sarcoglycan-deficient (Sgcg(-/-)) mouse model of limb-girdle muscular dystrophy (LGMD) 2C to antibody-mediated myostatin blockade in vivo. Myostatin inhibition led to increased fiber size, muscle mass, and absolute force. However, no clear improvement in muscle histopathology was evident, demonstrating discordance between physiological and histological improvement. These results and previous studies on the dyw/dyw mouse model of congenital muscular dystrophy and in the late-stage delta-sarcoglycan-deficient (Sgcd(-/-)) mouse model of LGMD2F document disease-specific limitations to therapeutic strategies based on myostatin blockade in the more severe mouse models of different muscular dystrophies.

译文

:肌生长抑制素是骨骼肌生长的负调节剂。肌肉生长抑制素的突变和药理学方法会增加体内肌肉的质量,这表明肌肉生长抑制素的阻断可能在以肌肉萎缩为特征的疾病(如肌肉营养不良)中被证明是有用的。我们经历了肢带肌肉萎缩症(LGMD)2C的γ-肌糖蛋白缺乏(Sgcg(-/-))小鼠模型体内抗体介导的肌生长抑制素阻断作用。抑制肌生长抑制素导致纤维大小,肌肉质量和绝对力增加。但是,肌肉组织病理学没有明显改善,表明生理和组织学改善之间不一致。这些结果和关于先天性肌营养不良的dyw / dyw小鼠模型以及LGMD2F的晚期δ-肌糖缺乏症(Sgcd(-/-))小鼠模型的先前研究记录了基于肌肉生长抑制素的疾病特异性治疗策略的局限性在不同肌肉营养不良的更严重的小鼠模型中具有阻断作用。

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