中国当代儿科杂志
中國噹代兒科雜誌
중국당대인과잡지
CHINA JOURNAL OF CONTEMPORARY PEDIATRICS
2004年
4期
256-260
,共5页
孙桂莲%姜红堃%胡宛如%获野谷和裕%饭沼一宇
孫桂蓮%薑紅堃%鬍宛如%穫野穀和裕%飯沼一宇
손계련%강홍곤%호완여%획야곡화유%반소일우
肌营养不良%金属蛋白酶组织抑制剂%转化生长因子-β1
肌營養不良%金屬蛋白酶組織抑製劑%轉化生長因子-β1
기영양불량%금속단백매조직억제제%전화생장인자-β1
Muscular dystrophy%Tissue inhibitor of metalloproteinases%Transforming growth factor-β1
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122.52±63.87 ng/ml,在先天性肌营养不良(CMD)为124.87±63.14 ng/ml,较对照组(85.71±29.13 ng/ml)明显升高(均P＜0.05),但在Becker型肌营养不良(BMD)为86.93±48.93 ng/ml,与对照组比较无明显升高;血浆TGF-β1水平在DMD为26.26±5.79 ng/ml,CMD为31.35±9.77 ng/ml,也较对照组(6.24±1.12 ng/ml)明显升高,差异有显著性,均P＜0.05,但在BMD为3.46±1.38 ng/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.
目的伴隨著肌纖維變性-再生的肌內異常結締組織增生為肌營養不良的一箇特徵.金屬蛋白酶組織抑製劑(TIMPs)是多功能蛋白,能改變細胞活性和調節基質轉變.轉化生長因子-β1(TGF-β1)也促進組織纖維化.該文探討TIMP-1和TGF-β1在各種肌營養不良髮病機製中的作用.方法用ELISA法檢測幾種肌營養不良患者血漿TIMP-1和TGF-β1水平.結果Duchenne肌營養不良(DMD)血漿TIMP-1水平為122.52±63.87 ng/ml,在先天性肌營養不良(CMD)為124.87±63.14 ng/ml,較對照組(85.71±29.13 ng/ml)明顯升高(均P＜0.05),但在Becker型肌營養不良(BMD)為86.93±48.93 ng/ml,與對照組比較無明顯升高;血漿TGF-β1水平在DMD為26.26±5.79 ng/ml,CMD為31.35±9.77 ng/ml,也較對照組(6.24±1.12 ng/ml)明顯升高,差異有顯著性,均P＜0.05,但在BMD為3.46±1.38 ng/ml,無升高;而且TIMP-1和TGF-β1濃度有明顯的相關性.結論血漿TIMP-1和TGF-β1水平在DMD和CMD中明顯升高,而BMD中未見明顯升高,似乎與肌營養不良的臨床嚴重性相關,錶明TIMP-1和TGF-β1在肌營養不良的髮病中可能起作用.
목적반수착기섬유변성-재생적기내이상결체조직증생위기영양불량적일개특정.금속단백매조직억제제(TIMPs)시다공능단백,능개변세포활성화조절기질전변.전화생장인자-β1(TGF-β1)야촉진조직섬유화.해문탐토TIMP-1화TGF-β1재각충기영양불량발병궤제중적작용.방법용ELISA법검측궤충기영양불량환자혈장TIMP-1화TGF-β1수평.결과Duchenne기영양불량(DMD)혈장TIMP-1수평위122.52±63.87 ng/ml,재선천성기영양불량(CMD)위124.87±63.14 ng/ml,교대조조(85.71±29.13 ng/ml)명현승고(균P＜0.05),단재Becker형기영양불량(BMD)위86.93±48.93 ng/ml,여대조조비교무명현승고;혈장TGF-β1수평재DMD위26.26±5.79 ng/ml,CMD위31.35±9.77 ng/ml,야교대조조(6.24±1.12 ng/ml)명현승고,차이유현저성,균P＜0.05,단재BMD위3.46±1.38 ng/ml,무승고;이차TIMP-1화TGF-β1농도유명현적상관성.결론혈장TIMP-1화TGF-β1수평재DMD화CMD중명현승고,이BMD중미견명현승고,사호여기영양불량적림상엄중성상관,표명TIMP-1화TGF-β1재기영양불량적발병중가능기작용.
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 levds 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-1level 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 devated 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 devation 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.
