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转化生长因子-β1在营养不良患者肌肉中的表达与纤维化相关。一种促纤维化细胞因子的致病作用。

Expression of transforming growth factor-beta 1 in dystrophic patient muscles correlates with fibrosis. Pathogenetic role of a fibrogenic cytokine.

作者信息

Bernasconi P, Torchiana E, Confalonieri P, Brugnoni R, Barresi R, Mora M, Cornelio F, Morandi L, Mantegazza R

机构信息

Department of Neuromuscular Diseases C. Besta National Neurological Institute, Milan, Italy.

出版信息

J Clin Invest. 1995 Aug;96(2):1137-44. doi: 10.1172/JCI118101.

DOI:10.1172/JCI118101
PMID:7635950
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC185304/
Abstract

Duchenne muscular dystrophy is a fatal disorder characterized by progressive muscular weakness, wasting, and severe muscle contractures in later disease stages. Muscle biopsy reveals conspicuous myofiber degeneration and fibrosis substituting muscle tissue. We quantitatively determined mRNA of the potent fibrogenic cytokine transforming growth factor-beta 1 by quantitative PCR in 15 Duchenne muscular dystrophy, 13 Becker muscular dystrophy, 11 spinal muscular atrophy patients, and 16 controls. Higher transforming growth factor-beta 1 expression was greater in Duchenne muscular dystrophy patients than controls (P = 0.012) and Becker patients (P = 0.03). Fibrosis was significantly more prominent in Duchenne muscular dystrophy than Becker muscular dystrophy, spinal muscular atrophy, and controls. The proportion of connective tissue in muscle biopsies increased progressively with age in Duchenne muscular dystrophy patients, while transforming growth factor-beta 1 levels peaked at 2 and 6 yr of age. Transforming growth factor-beta 1 protein was also detected by immunocytochemistry and immunoblotting. Our findings suggest that transforming growth factor-beta 1 stimulates fibrosis in Duchenne muscular dystrophy. Expression of transforming growth factor-beta 1 in the early stages of Duchenne muscular dystrophy may be critical in initiating muscle fibrosis and antifibrosis treatment could slow progression of the disease, increasing the utility of gene therapy.

摘要

杜氏肌营养不良症是一种致命性疾病，其特征为进行性肌肉无力、肌肉萎缩，在疾病后期会出现严重的肌肉挛缩。肌肉活检显示明显的肌纤维变性以及肌肉组织被纤维组织替代。我们通过定量聚合酶链反应对15例杜氏肌营养不良症患者、13例贝克型肌营养不良症患者、11例脊髓性肌萎缩症患者和16名对照者的强效促纤维化细胞因子转化生长因子-β1的信使核糖核酸进行了定量测定。杜氏肌营养不良症患者的转化生长因子-β1表达高于对照者（P = 0.012）和贝克型肌营养不良症患者（P = 0.03）。杜氏肌营养不良症中的纤维化比贝克型肌营养不良症、脊髓性肌萎缩症及对照者更为显著。在杜氏肌营养不良症患者中，肌肉活检中结缔组织的比例随年龄逐渐增加，而转化生长因子-β1水平在2岁和6岁时达到峰值。还通过免疫细胞化学和免疫印迹法检测到了转化生长因子-β1蛋白。我们的研究结果表明，转化生长因子-β1在杜氏肌营养不良症中刺激纤维化。杜氏肌营养不良症早期转化生长因子-β1的表达可能对引发肌肉纤维化至关重要，抗纤维化治疗可能会减缓疾病进展，从而增加基因治疗的效用。

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