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Histological changes in masticatory muscles of mdx mice.

机译:mdx小鼠咀嚼肌的组织学变化。

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OBJECTIVE: Duchenne muscular dystrophy (DMD) patients have distorted dentofacial morphology that could be a result of changed force balance of masticatory muscles due to unequal dystrophic changes in various masticatory muscles. Skeletal muscles of DMD patients and those of murine model of DMD - mdx mice - are both characterized by Ca(2+) induced muscle damage, muscle weakness and characteristic histological changes. Therefore, to determine the pathological changes in this animal model of DMD, we examined the masticatory muscles of the mdx mice for histological abnormalities including nuclei localization, fibre diameters, and collagen expression. DESIGN: Muscle sections from masseter (MAS), temporal (TEM), tongue (TON) and soleus (SOL) of mdx and control normal mice were stained with hemalaun/eosin or with Sirius Red and morphometrically analysed. Levels of collagen staining in normal and mdx muscles were measured using image analysis and the mean optical density (mod) was determined. RESULTS: Dystrophin deficient masticatory muscles contained 11-75% fibres with centralised nuclei. In mdx mice an increased mean fibre diameter was observed as compared to the age-matched control muscles (control vs. mdx; MAS: 33.44+/-0.49microm vs. 37.76+/-0.68microm, p<0.005; TEM: 32.93+/-0.4microm vs. 42.93+/-0.68microm, p<0.005; SOL: 33.15+/-0.29microm vs. 40.62+/-0.55microm, p<0.005; TON: 13.44+/-0.68microm vs. 15.63+/-0.18microm, p<0.005). Increased expression of collagen was found in MAS (mod control vs. mdx: 1.34 vs. 3.99, p<0.005), TEM (mod control vs. mdx: 3.11 vs. 4.73, p<0.01) and SOL (mod control vs. mdx: 2.36 vs. 3.49, p<0.01). CONCLUSION: Our findings revealed that mdx masticatory muscles are unequally affected by the disease process. The masticatory muscles of the mdx mice could present a useful model for further investigating the influence of dystrophin deficiency on muscles function.
机译:目的:杜兴氏肌营养不良症(DMD)患者的牙齿面形畸变,可能是由于咀嚼肌的营养不良变化不均等导致咀嚼肌力平衡改变的结果。 DMD患者的骨骼肌和DMD的鼠模型-mdx小鼠的骨骼肌均以Ca(2+)诱导的肌肉损伤,肌肉无力和特征性组织学改变为特征。因此,为了确定这种DMD动物模型的病理变化,我们检查了mdx小鼠咀嚼肌的组织学异常,包括细胞核定位,纤维直径和胶原蛋白表达。设计:用hemalaun /伊红或Sirius Red对mdx和对照正常小鼠的咬肌(MAS),颞骨(TEM),舌头(TON)和比目鱼肌(SOL)的肌肉切片进行染色,并进行形态分析。使用图像分析测量正常和mdx肌肉的胶原蛋白染色水平,并确定平均光密度(mod)。结果:肌营养不良蛋白缺乏的咀嚼肌含有11-75%的纤维,具有集中的核。与年龄匹配的对照肌肉相比,在mdx小鼠中观察到平均纤维直径增加(对照vs.mdx; MAS:33.44 +/- 0.49microm vs.37.76 +/- 0.68microm,p <0.005; TEM:32.93+ /-0.4微米对42.93 +/- 0.68微米,p <0.005; SOL:33.15 +/- 0.29微米对40.62 +/- 0.55微米,p <0.005; TON:13.44 +/- 0.68微米对15.63+ /-0.18微米,p <0.005)。在MAS(mod对照vs.mdx:1.34 vs. 3.99,p <0.005),TEM(mod对照vs.mdx:3.11 vs.4.73,p <0.01)和SOL(mod对照vs.mdx)中发现胶原蛋白表达增加:2.36 vs.3.49,p <0.01)。结论:我们的发现表明,mdx咀嚼肌受到疾病过程的不平等影响。 mdx小鼠的咀嚼肌可能会提供一个有用的模型,用于进一步研究肌营养不良蛋白缺乏对肌肉功能的影响。

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