Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK
National Institute for Health Research, Great Ormond Street Institute of Child Health Biomedical Research Centre, University College London, London WC1N 1EH, UK.
Dis Model Mech. 2020 Feb 6;13(2):dmm042192. doi: 10.1242/dmm.042192.
Skeletal muscle fibres are multinucleated cells that contain postmitotic nuclei (i.e. they are no longer able to divide) and perform muscle contraction. They are formed by fusion of muscle precursor cells, and grow into elongating myofibres by the addition of further precursor cells, called satellite cells, which are also responsible for regeneration following injury. Skeletal muscle regeneration occurs in most muscular dystrophies in response to necrosis of muscle fibres. However, the complex environment within dystrophic skeletal muscle, which includes inflammatory cells, fibroblasts and fibro-adipogenic cells, together with the genetic background of the model and the muscle being studied, complicates the interpretation of laboratory studies on muscular dystrophies. Many genes are expressed in satellite cells and in other tissues, which makes it difficult to determine the molecular cause of various types of muscular dystrophies. Here, and in the accompanying poster, we discuss our current knowledge of the cellular mechanisms that govern the growth and regeneration of skeletal muscle, and highlight the defects in satellite cell function that give rise to muscular dystrophies.
骨骼肌纤维是多核细胞,包含有丝分裂后核(即不再能够分裂),并执行肌肉收缩。它们是由肌肉前体细胞融合形成的,并通过进一步添加称为卫星细胞的前体细胞生长成伸长的肌纤维,卫星细胞还负责损伤后的再生。在大多数肌肉营养不良症中,骨骼肌再生是由于肌肉纤维坏死引起的。然而,在肌肉营养不良症的病变骨骼肌中存在复杂的环境,包括炎症细胞、成纤维细胞和成纤维脂肪细胞,以及模型和研究肌肉的遗传背景,这使得对肌肉营养不良症的实验室研究的解释变得复杂。许多基因在卫星细胞和其他组织中表达,这使得确定各种类型肌肉营养不良症的分子原因变得困难。在这里,以及在相关海报中,我们讨论了我们目前对支配骨骼肌生长和再生的细胞机制的了解,并强调了导致肌肉营养不良症的卫星细胞功能缺陷。