Department of Immunobiology, Fluminense Federal University, Niterói, RJ 24 020 141, Brazil.
Cell Tissue Res. 2012 Oct;350(1):77-88. doi: 10.1007/s00441-012-1443-3. Epub 2012 Jun 13.
The mdx (X chromosome-linked muscular dystrophy) mouse develops a multi-staged disorder characterized by muscle degeneration and reactive fibrosis. Skeletal muscles of mdx mice are not equally susceptible to degeneration. The aim of this study was to verify whether the intense remodeling of the mdx diaphragm could be attributed to influences from the peritoneal microenvironment and omentum, a lymphohematopoietic tissue rich in progenitor cells and trophic factors. At ages corresponding to increased muscular regeneration (12 weeks) and activation of fibrosis (24 weeks), the mdx omentum exhibited (1) morphological and functional characteristics of activation with enlarged milk-spots, an accumulation of CD4(+), CD8(+) and CD19(+)B220(+) B lymphocytes; (2) the formation of clusters positive for proliferating cell nuclear antigen, mainly in B220(+)-rich areas organized in a follicular structure with a germinative center without any challenge by external antigen inducers; (3) clusters with cells positive for fibroblast growth factor-2, numerous Sca-1(+)CD3(-)CD19(-)Mac-1(-) progenitor cells and increased CD4(+), CD8(+) and CD3(+)NK1.1(+) cells in the peritoneal cavity. Omentectomy reduced areas with F4/80(+) inflammatory infiltrate the activity of matrix metalloproteases 9 and 2, collagen deposition and areas with regenerating myofibers in the diaphragm. Thus, persistent activation of the omentum influences the pattern of inflammation and regeneration of the mdx diaphragm partly via the activation of progenitor cells and the production of growth factors that influence the physiopathology of the muscular tissue remodeling.
mdx(X 染色体连锁肌肉营养不良症)小鼠表现出多阶段的疾病特征,包括肌肉退化和反应性纤维化。mdx 小鼠的骨骼肌对退化的敏感性并不相同。本研究的目的是验证 mdx 膈肌的强烈重塑是否归因于腹膜微环境和大网膜的影响,大网膜是富含祖细胞和营养因子的淋巴造血组织。在肌肉再生增加(12 周)和纤维化激活(24 周)对应的年龄,mdx 大网膜表现出(1)形态和功能激活特征,表现为乳斑增大,CD4(+)、CD8(+)和 CD19(+)B220(+)B 淋巴细胞积聚;(2)增殖细胞核抗原阳性的簇形成,主要在富含 B220(+)的区域形成滤泡结构,具有生发中心,但没有任何外源性抗原诱导物的挑战;(3)成纤维细胞生长因子-2 阳性簇,大量 Sca-1(+)CD3(-)CD19(-)Mac-1(-)祖细胞,腹腔中 CD4(+)、CD8(+)和 CD3(+)NK1.1(+)细胞增加。大网膜切除术减少了膈肌中 F4/80(+)炎症浸润、基质金属蛋白酶 9 和 2 的活性、胶原蛋白沉积和再生肌纤维的区域。因此,大网膜的持续激活通过激活祖细胞和产生影响肌肉组织重塑生理病理学的生长因子,影响 mdx 膈肌的炎症和再生模式。