mdx 和 mdx/utrn(+/-) 小鼠呼吸功能受损。
Impaired respiratory function in mdx and mdx/utrn(+/-) mice.
机构信息
Department of Neurology, Cleveland Clinic, Cleveland, OH 44195, USA.
出版信息
Muscle Nerve. 2011 Feb;43(2):263-7. doi: 10.1002/mus.21848.
Abstract
Muscle fibrosis is a prominent pathological feature that directly causes muscle dysfunction in Duchenne muscular dystrophy (DMD). The DMD mouse models, mdx mice and mdx mice with haploinsufficiency of the utrophin gene (mdx/utrn(+/-) ), display progressive diaphragm fibrosis. We performed unrestrained whole-body plethysmography (WBP) in mdx and mdx/utrn(+/-) mice, and compared them with wild-type controls. Respiratory function gauged by respiratory frequency, tidal volume, minute volume, peak inspiratory flow, and peak expiratory flow was significantly impaired in the mdx mice. Consistent with more severe diaphragm fibrosis in the mdx/utrn(+/-) mice, respiratory impairment was worse than in mdx mice at 6 months. WBP is useful for monitoring in vivo respiratory function of mdx and mdx/utrn(+/-) mice, and it may serve as an outcome measurement for therapies that target diaphragm fibrosis. The mdx/utrn(+/-) mouse model may be better than the mdx model for testing antifibrotic therapies, especially at the severe stage.
摘要
肌肉纤维化是杜氏肌营养不良症（DMD）中直接导致肌肉功能障碍的主要病理学特征。mdx 小鼠和 utrophin 基因杂合缺失（mdx/utrn(+/-)）的 mdx 小鼠模型表现出进行性膈肌纤维化。我们对 mdx 和 mdx/utrn(+/-)小鼠进行了非约束性全身呼吸描记术（WBP），并与野生型对照进行了比较。呼吸频率、潮气量、分钟通气量、吸气峰流速和呼气峰流速等指标表明，mdx 小鼠的呼吸功能明显受损。与 mdx/utrn(+/-)小鼠更严重的膈肌纤维化一致，呼吸损伤在 6 个月时比 mdx 小鼠更严重。WBP 可用于监测 mdx 和 mdx/utrn(+/-)小鼠的体内呼吸功能，并且可能成为针对膈肌纤维化的治疗方法的疗效评估指标。与 mdx 模型相比，mdx/utrn(+/-)小鼠模型在测试抗纤维化治疗方法，特别是在严重阶段时可能更优。
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