P2X7 activation enhances skeletal muscle metabolism and regeneration in SOD1G93A mouse model of amyotrophic lateral sclerosis
作者: Paola FabbrizioSavina ApolloniAndrea BianchiIllari SalvatoriCristiana ValleChiara LanzuoloCaterina BendottiGiovanni NardoCinzia Volonté
作者单位: 1Laboratory of Molecular Neurobiology, Department of Neuroscience Istituto di Ricerche Farmacologiche Mario Negri IRCCS Milan Italy
2 IRCCS Fondazione Santa Lucia Rome Italy
3 National Research Council, Institute of Translational Pharmacology Rome Italy
4 National Research Council, Institute of Biomedical Technologies Milan Italy
5 National Research Council, Institute for Systems Analysis and Computer Science Rome Italy
刊名: Brain Pathology, 2020, Vol.30 (2), pp.272-282
来源数据库: Wiley Journal
DOI: 10.1111/bpa.12774
关键词: Amyotrophic lateral sclerosisPurinergic receptorsSkeletal muscleSOD1G93A mice
原始语种摘要: Abstract(#br)Muscle weakness plays an important role in neuromuscular disorders comprising amyotrophic lateral sclerosis (ALS). However, it is not established whether muscle denervation originates from the motor neurons, the muscles or more likely both. Previous studies have shown that the expression of the SOD1G93A mutation in skeletal muscles causes denervation of the neuromuscular junctions, inability to regenerate and consequent atrophy, all clear symptoms of ALS. In this work, we used SOD1G93A mice, a model that best mimics some pathological features of both familial and sporadic ALS, and we investigated some biological effects induced by the activation of the P2X7 receptor in the skeletal muscles. The P2X7, belonging to the ionotropic family of purinergic receptors for extracellular...
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关键词翻译
关键词翻译
  • muscles 筋肉
  • skeletal 骨架
  • sclerosis 硬化
  • SOD Superintendent Of Documents
  • amyotrophic 肌肉萎缩的
  • metabolism 代谢
  • regeneration 再生
  • activation 活化
  • mouse 小鼠
  • neuromuscular 神经肌肉