Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration

摘要: Selective motor neuron degeneration is a hallmark of amyotrophic lateral sclerosis (ALS). Around 10% all cases present as familial ALS (FALS), while sporadic (SALS) accounts for the remaining 90%. Diverse genetic mutations leading to FALS have been identified, but underlying causes SALS remain largely unknown. Despite heterogeneous and incompletely understood etiology, different types exhibit overlapping pathology common phenotypes, including protein aggregation mitochondrial deficiencies. Here, we review current understanding mechanisms in they pertain disrupted cellular clearance pathways, ATP biogenesis, calcium buffering dynamics. Through focusing on impaired autophagic functions, highlight how convergence diverse processes pathways contributes degeneration.