Mdx respiratory impairment following fibrosis of the diaphragm
Mdx respiratory impairment following fibrosis of the diaphragm
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DOI：
10.1016/j.nmd.2008.02.002
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发表时间：
2008-04-01
影响因子：
2.8
通讯作者：
Uchino, Makoto
中科院分区：
文献类型：
--
作者：
Ishizaki, Masatoshi;Suga, Tomohiro;Uchino, Makoto
Duchenne肌营养不良症(DMD)是一种进行性肌肉萎缩疾病，会导致呼吸或心脏衰竭，并导致大约20岁的死亡。DMD的动物模型MDX小鼠通常被用来评估营养不良的病理。四肢肌肉的病理特征相对较轻，但横隔肌受到严重影响，并呈现出与人类DMD相似的退行性变化模式。虽然，营养不良的横隔肌的肌力分析已经被用来评估MDX呼吸损害，但与组织病理学分析相比，系统功能评估还没有得到证实。在这里，我们报告了一种使用全身体积描记术监测MDX小鼠早期呼吸功能不全期间检测到的呼吸参数的灵敏程序。隔膜的营养不良改变会导致呼吸功能障碍。这些方法可能有助于评估MDX小鼠的治疗方法。(C)2008爱思唯尔B.V.保留所有权利。
Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disease that causes respiratory or cardiac failure and results in death at about 20 years of age. An animal model of DMD, the mdx mouse, is commonly used to estimate dystrophic pathology. The pathological features of limb muscles are relatively mild, however the diaphragm is severely affected and exhibits a degenerative pattern similar to that observed in human DMD. Although, the muscle strength assay of the dystrophic diaphragm has been used to estimate mdx respiratory impairment, systemic functional assessments compared with histopathological analysis have not been demonstrated. Here, we report a sensitive procedure using whole-body plethysmography to monitor respiratory parameters detected during early respiratory insufficiency in the mdx mouse. The dystrophic changes in the diaphragm lead to respiratory dysfunctions. These methods may be useful to assess the therapeutic approaches for the mdx mouse. (C) 2008 Elsevier B.V. All rights reserved.