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目的 伴随着肌纤维变性 再生的肌内异常结缔组织增生为肌营养不良的一个特征。金属蛋白酶组织抑制剂 (TIMPs)是多功能蛋白 ,能改变细胞活性和调节基质转变。转化生长因子 β1(TGF β1)也促进组织纤维化。该文探讨TIMP 1和TGF β1在各种肌营养不良发病机制中的作用。 方法 用ELISA法检测几种肌营养不良患者血浆TIMP 1和TGF β1水平。 结果 Duchenne肌营养不良 (DMD)血浆TIMP 1水平为 12 2 .5 2±6 3.87ng/ml,在先天性肌营养不良 (CMD)为 12 4 .87± 6 3.14ng/ml,较对照组 (85 .71± 2 9.13ng/ml)明显升高 (均P <0 .0 5 ) ,但在Becker型肌营养不良 (BMD)为 86 .93± 4 8.93ng/ml,与对照组比较无明显升高 ;血浆TGF β1水平在DMD为 2 6 .2 6± 5 .79ng/ml,CMD为 31.35± 9.77ng/ml,也较对照组 (6 .2 4± 1.12ng/ml)明显升高 ,差异有显著性 ,均P <0 .0 5 ,但在BMD为 3.4 6± 1.38ng/ml,无升高 ;而且TIMP 1和TGF β1浓度有明显的相关性。结论 血浆TIMP 1和TGF β1水平在DMD和CMD中明显升高 ,而BMD中未见明显升高 ,似乎与肌营养不良的临床严重性相关 ,表明TIMP 1和TGF β1在肌营养不良的发病中可能起作用。
Purpose A myotrophic dystrophy is a characteristic of intramuscular abnormal connective tissue hyperplasia associated with myofibrillar regeneration. Metalloproteinase inhibitors (TIMPs) are multifunctional proteins that alter cellular activity and modulate matrix turnover. Transforming growth factor β1 (TGFβ1) also promotes tissue fibrosis. This article explores the role of TIMP-1 and TGFpi in the pathogenesis of various muscular dystrophy. Methods Plasma levels of TIMP-1 and TGF-β1 in several muscular dystrophy patients were measured by ELISA. Results The plasma levels of TIMP-1 in Duchenne muscular dystrophy (DMD) were 12 2 .5 2 ± 6 3.87 ng / ml and in congenital muscular dystrophy (CMD) 12 4 .87 ± 6 3.14 ng / ml, 85.71 ± 2 9.13ng / ml) (all P <0.05), but there was no significant difference in Becker muscular dystrophy (BMD) of 86.93 ± 4.893ng / ml compared with the control group (P <0.05). The level of plasma TGF-β1 in DMD was 26.66 ± 5.79ng / ml and CMD was 31.35 ± 9.77ng / ml, which was also significantly higher than that in control group (6.24 ± 1.12ng / ml) There was significant difference between the two groups (P <0.05), but there was no increase in BMD of 3.4 6 ± 1.38ng / ml. There was a significant correlation between TIMP-1 and TGF-β1 concentrations. Conclusions The levels of plasma TIMP-1 and TGF-β1 were significantly increased in DMD and CMD but not in BMD, which seemed to be related to the clinical severity of muscular dystrophy, suggesting that TIMP-1 and TGF-β1 are involved in the pathogenesis of muscular dystrophy May work.