Floss T, Arnold H H, Braun T
Department of Cell and Molecular Biology, University of Braunschweig, Germany.
Genes Dev. 1997 Aug 15;11(16):2040-51. doi: 10.1101/gad.11.16.2040.
Fibroblast growth factor-6 (FGF-6) belongs to a family of cytokines that control cell proliferation, cell differentiation, and morphogenetic events. Individual FGFs are either expressed widely or in a restricted pattern during embryonic, fetal, and adult life. FGF-6 exhibits a restricted expression profile predominantly in the myogenic lineage. Important functions in wound healing and tissue regeneration have been proposed for various FGFs in the past, although data from knockout mice have not supported this view. We have inactivated the FGF-6 gene in mice to investigate the role of FGF-6 in skeletal muscle development and regeneration. Wild-type mice up-regulate FGF-6 after skeletal muscle injuries and completely restore experimentally damaged skeletal muscle. In contrast, FGF-6(-/-) mutant mice show a severe regeneration defect with fibrosis and myotube degeneration. The number of MyoD- and Myogenin-expressing activated satellite cells after injury were significantly reduced in mutants. This reduction was not caused by a reduced pool of quiescent satellite cells but presumably by a lack of activation or proliferation. Interbreeding of FGF-6(-/-) mutants with mdx mice leads to striking dystrophic changes in skeletal muscles of double homozygous mice characterized by myotube degeneration, the presence of large amounts of mononuclear cells, and deposition of collagen. RNA analysis revealed an up-regulation of MyoD mRNA in mdx but not in FGF-6(-/-)/mdx double mutant mice. We conclude that FGF-6 is a critical component of the muscle regeneration machinery in mammals, possibly by stimulating or activating satellite cells.
成纤维细胞生长因子-6(FGF-6)属于一类控制细胞增殖、细胞分化和形态发生事件的细胞因子家族。在胚胎期、胎儿期和成年期,单个成纤维细胞生长因子要么广泛表达,要么以受限模式表达。FGF-6主要在肌源性谱系中呈现受限的表达谱。过去曾提出各种成纤维细胞生长因子在伤口愈合和组织再生中具有重要功能,尽管基因敲除小鼠的数据并不支持这一观点。我们已使小鼠体内的FGF-6基因失活,以研究FGF-6在骨骼肌发育和再生中的作用。野生型小鼠在骨骼肌损伤后会上调FGF-6,并能完全修复实验性损伤的骨骼肌。相比之下,FGF-6(-/-)突变小鼠表现出严重的再生缺陷,伴有纤维化和肌管退化。突变体中损伤后表达MyoD和生肌调节因子的活化卫星细胞数量显著减少。这种减少不是由静止卫星细胞池减少引起的,而是可能由于缺乏激活或增殖。FGF-6(-/-)突变体与mdx小鼠杂交导致双纯合小鼠骨骼肌出现明显的营养不良性变化,其特征为肌管退化、大量单核细胞的存在和胶原蛋白沉积。RNA分析显示,mdx小鼠中MyoD mRNA上调,但FGF-6(-/-)/mdx双突变小鼠中未上调。我们得出结论,FGF-6可能通过刺激或激活卫星细胞,是哺乳动物肌肉再生机制的关键组成部分。