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肌球蛋白重链同工型在营养不良蛋白缺陷小鼠咀嚼肌中的差异表达。

Differential expression of myosin heavy chain isoforms in the masticatory muscles of dystrophin-deficient mice.

机构信息

Department of Orthodontics, Faculty of Medicine, University of Greifswald, Germany.

出版信息

Eur J Orthod. 2011 Dec;33(6):613-9. doi: 10.1093/ejo/cjq113. Epub 2010 Dec 27.

Abstract

The dystrophin-deficient mouse (mdx) is a homologue animal model of Duchenne muscular dystrophy (DMD) and is characterized by slowly progressive muscle weakness accompanied by changes in myosin heavy chain (MyHC) composition. It is likely that the masticatory muscles undergo similar changes. The aim of this study was to examine the masticatory muscles (masseter, temporal, tongue, and soleus) of 100-day-old mdx and control mice (n = 8-10), and the fibre type distribution (by immunohistochemistry) as well as the expression of the corresponding MyHC messenger RNA (mRNA) (protein and mRNA expression, using Western blot or quantitative real-time polymerase chain reaction (RT-PCR)). Immunohistochemistry and western blot analysis revealed that the masticatory muscles in the control and mdx mice consisted mainly of type 2 fibres, whereas soleus muscle consisted of both type 1 and 2 fibres. In the masseter muscle, the mRNA in mdx mice was not different from that found in the controls. However, the mRNA content of the MyHC-2b isoform in mdx mice was lower in comparison with the controls in the temporal muscle [11.9 versus 36.9 per cent; P < 0.01; mean ± standard error of the mean (SEM), Student's unpaired t-test], as well as in the tongue muscle (65.7 versus 73.8 per cent; P < 0.05). Similarly, the content of MyHC-2x isoforms in mdx tongue muscle was lower than in the controls (25.9 versus 30.8 per cent; P < 0.05). The observed down-regulation of the MyHC-2x and MyHC-2b mRNA in the masticatory muscles of mdx mice may lead to changed fibre type composition. The different MyHC gene expression in mdx mice masticatory muscles may be seen as an adaptive mechanism to muscular dystrophy.

摘要

营养不良型肌萎缩症(DMD)缺陷小鼠(mdx)是杜兴氏肌肉营养不良症(DMD)的同源动物模型,其特征是肌肉无力进行性缓慢进展,同时肌球蛋白重链(MyHC)组成发生变化。咀嚼肌很可能也发生类似的变化。本研究旨在检查 100 日龄 mdx 和对照小鼠(n = 8-10)的咀嚼肌(咬肌、颞肌、舌肌和比目鱼肌),以及纤维类型分布(免疫组织化学)以及相应的 MyHC 信使 RNA(mRNA)的表达(使用 Western blot 或定量实时聚合酶链反应(RT-PCR))。免疫组织化学和 Western blot 分析显示,对照和 mdx 小鼠的咀嚼肌主要由 2 型纤维组成,而比目鱼肌既有 1 型纤维又有 2 型纤维。在咬肌中,mdx 小鼠的 mRNA 与对照组无差异。然而,mdx 小鼠颞肌中的 MyHC-2b 同工型 mRNA 含量明显低于对照组[11.9%比 36.9%;P < 0.01;学生无配对 t 检验,均值 ± 标准误],舌肌中也低于对照组[65.7%比 73.8%;P < 0.05]。同样,mdx 舌肌中的 MyHC-2x 同工型含量也低于对照组[25.9%比 30.8%;P < 0.05]。mdx 小鼠咀嚼肌中 MyHC-2x 和 MyHC-2b mRNA 的下调可能导致纤维类型组成发生变化。mdx 小鼠咀嚼肌中不同的 MyHC 基因表达可能被视为对肌肉营养不良的一种适应机制。

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