Autophagic dysfunction in amyotrophic lateral sclerosis

• 作 者: Xiao-Jie ZHANG Liang LI Sheng CHEN Wei-Dong LE
• 机 构: Neurogenomics, Institute of Health Sciences, SIBS, CAS-SJTUSM, Shanghai 200025, China
• 出 处: 中国神经科学学会第九届全国学术会议暨第五届会员代表大会
• 发表日期: 2011年1期
• 关键词: ALS motor neuron degeneration protein aggregation autophagy apoptosis

　　Aberrant protein misfolding may contribute to the pathogenesis of amyotrophic lateral sclerosis (ALS) but the detailed mechanisms are largely unknown.To investigate if there is any autophagic alteration in ALS and used SOD1G93A mutant mice, a model of ALS to examined the processings of autophgy related molecules (LC3, Beclin-l, p-mTOR, p62, p-P70, p62, Atg5 and Atgl2 processing) by immunostaining and Western blot in the spinal cord and other central nervous system.We have demonstrated that the autophagic protein marker LC3-Ⅱ is markedly and specifically increased in the spinal cord motor neurons (MNs) of the ALS mice.Electron microscopy and immunochemistry studies have shown that autophagic vacuoles are significantly accumulated in the dystrophic axons of spinal cord MNs of the ALS mice.