Departamento de Anatomia, Biologia Celular, Fisiologia e Biofísica, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, SãoPaulo 13083-970, Brazil.
Muscle Nerve. 2011 Jan;43(1):82-7. doi: 10.1002/mus.21869.
Fibrosis is a pathological feature observed in patients with Duchenne muscular dystrophy (DMD) and in mdx mice, the experimental model of DMD. We evaluated the effect of suramin, a transforming growth factor-beta 1 (TGF-β1) blocker, on fibrosis in mdx mice. mdx mice (6 months old) received suramin for 7 weeks. Suramin- and saline-treated (control) mdx mice performed exercise on a treadmill to worsen disease progression. Immunoblotting showed an increase of TGF-β1 in mdx diaphragm, limb, and cardiac muscles. Suramin decreased creatine kinase in mdx mice and attenuated fibrosis in all muscles studied, except for cardiac muscle. Suramin protected limb muscles against damage and reduced the exercise-induced loss of strength over time. These findings support a role for TGF-β1 in fibrinogenesis and myonecrosis during the later stages of disease in mdx mice. Suramin might be a useful therapeutic alternative for the treatment of dystrophinopathies.
纤维化是杜兴氏肌营养不良症（DMD）患者和 mdx 小鼠（DMD 的实验模型）中观察到的一种病理特征。我们评估了苏拉明（一种转化生长因子-β1（TGF-β1）阻滞剂）对 mdx 小鼠纤维化的影响。6 月龄 mdx 小鼠接受苏拉明治疗 7 周。苏拉明和生理盐水处理（对照）的 mdx 小鼠在跑步机上进行运动以加重疾病进展。免疫印迹显示 mdx 膈肌、四肢和心肌中的 TGF-β1 增加。苏拉明降低了 mdx 小鼠的肌酸激酶，并减轻了所有研究肌肉的纤维化，心脏肌肉除外。苏拉明保护四肢肌肉免受损伤，并随着时间的推移减少运动引起的力量丧失。这些发现支持 TGF-β1 在 mdx 小鼠疾病后期肌纤维生成和肌坏死中的作用。苏拉明可能是治疗肌营养不良症的一种有用的治疗选择。
