T and B lymphocyte depletion has a marked effect on the fibrosis of dystrophic skeletal muscles in the scid/mdx mouse
T and B lymphocyte depletion has a marked effect on the fibrosis of dystrophic skeletal muscles in the scid/mdx mouse
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T 和 B 淋巴细胞耗竭对 scid/mdx 小鼠营养不良的骨骼肌纤维化有显着影响
作者：
A. Farini;M. Meregalli;M. Belicchi;M. Battistelli;D. Parolini;G. D’Antona;M. Gavina;L. Ottoboni;G. Constantin;R. Bottinelli;Y. Torrente
肌肉退化后异常结缔组织增殖是杜氏肌营养不良症 (DMD) 的主要病理特征，DMD 是一种由于缺乏肌膜肌营养不良蛋白而导致的遗传性肌病。由于这种纤维化增殖可能是未来治疗效果的主要障碍，因此需要进行研究来了解和预防纤维化过程，以便开发有效的治疗方法。小鼠肌营养不良症 (mdx) 在基因上与 DMD 是同源的，组织病理学改变与 DMD 患者的肌肉改变相当。为了研究纤维化的发展，我们将 mdx 小鼠与 scid 免疫抑制小鼠交配，并对纤维化进行组织学分析。我们使用 ELISA 分析来确定 TGF-β1 的表达。 scid/mdx 小鼠的肌肉纤维化和 TGF-β1 表达显着减少。然而，与 mdx 动物相比，我们观察到类似的位于中心的细胞核、坏死、肌肉变性和肌肉力量。这些数据表明，B 和 T 淋巴细胞的缺失与伴随 TGF-β1 减少的纤维化消失之间存在相关性，表明免疫系统调节在 DMD 中的重要性。版权所有 © 2007 大不列颠及爱尔兰病理学会。由约翰·威利父子有限公司出版
Abnormal connective tissue proliferation following muscle degeneration is a major pathological feature of Duchenne muscular dystrophy (DMD), a genetic myopathy due to lack of the sarcolemmal dystrophin protein. Since this fibrotic proliferation is likely to be a major obstacle to the efficacy of future therapies, research is needed to understand and prevent the fibrotic process in order to develop an effective treatment. Murine muscular dystrophy (mdx) is genetically homologous to DMD, and histopatological alterations are comparable to those of the muscles of patients with DMD. To investigate the development of fibrosis, we bred the mdx mouse with the scid immunodepressed mouse and analysed fibrosis histologically; we used ELISA analysis to determine TGF‐β1 expression. Significant reduction of fibrosis and TGF‐β1 expression was found in the muscles of the scid/mdx mice. However, we observed similar centrally located nuclei, necrosis, muscle degeneration and muscle force compared to the mdx animals. These data demonstrate a correlation between the absence of B and T lymphocytes and loss of fibrosis accompanied by reduction of TGF‐β1, suggesting the importance of modulation of the immune system in DMD. Copyright © 2007 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
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影响因子：
56.9
作者：
Campbell, JJ;Hedrick, J;Butcher, EC
通讯作者：
Butcher, EC
影响因子：
--
作者：
M. Barcellos-Hoff;T. Dix
通讯作者：
M. Barcellos-Hoff;T. Dix
影响因子：
--
作者：
FIELDING, RA;MANFREDI, TJ;CANNON, JG
通讯作者：
CANNON, JG
通过转化生长因子-β 和肿瘤坏死因子-α 调节人肺成纤维细胞 C1q 受体。
影响因子：
1.7
作者：
Lurton,J;Soto,H;Narayanan,AS;Raghu,G
通讯作者：
Raghu,G
影响因子：
3.3
作者：
ENGEL, AG;ARAHATA, K
通讯作者：
ARAHATA, K