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。

OBJECTIVE: Abnormal connective tissue proliferation in muscle following muscle fibre degeneration-regeneration is a feature of muscular dystrophy. Tissue inhibitors of metalloproteinases (TIMPs) are multifunctional proteins that can modify cellular activities and modulate matrix turnover. Transforming growth factor-β1 (TGF-β1) can promote tissue fibrosis. This paper studied the role of TIMP-1 and TGF-β1 in the pathogenesis of various muscular dystrophies. METHODS: Plasma TIMP-1 and TGF-β1 levels were measured by ELISA in patients with various muscular dystrophies. Forty-one patients with non-muscle disorders were used as the Control group. RESULTS: The plasma TIMP-1 level was significantly elevated in patients with Duchenne muscular dystrophy (DMD, 122.52± 63.87 ng/ml) (P< 0.05) and congenital muscular dystrophy (CMD, 124.87± 63.14 ng/ml) (P< 0.05) when compared with that of the Control group ( 85.71± 29.13 ng/ml). Patients with Becker muscular dystrophy (BMD) had no significant elevation of the TIMP-1 level compared with the Control group. Compared with the Control group ( 6.24± 1.12 ng/ml), the plasma TGF-β1 level was significantly elevated in patients with DMD ( 26.26± 5.79 ng/ml) (P< 0.01) and CMD ( 31.35± 9.77 ng/ml) (P< 0.05), but not in patients with BMD ( 3.46± 1.38 ng/ml). There was a correlation between the concentrations of TIMP-1 and TGF-β1 (r= 0.6350,P< 0.01). CONCLUSIONS: The plasma TIMP-1 and TGF-β1 levels were elevated in patients with DMD or CMD. This elevation suggests that TIMP-1 and TGF-β1 are correlated with the clinical severity of muscular dystrophy and suggests that they may play a role in the genesis of muscular dystrophy.[WT5"H