Amyotrophic lateral sclerosis (ALS) is a degenerative disorder that is characterized by loss of motor neurons and shows clinical, pathological, and genetic overlap with frontotemporal dementia (FTD). Activated microglia are a universal feature of ALS/FTD pathology; however, their role in disease pathogenesis remains incompletely understood. The recent discovery that ORF 72 on chromosome 9 (C9orf72), the gene most commonly mutated in ALS/FTD, has an important role in myeloid cells opened the possibility that altered microglial function plays an active role in disease. This Review highlights the contribution of microglia to ALS/FTD pathogenesis, discusses the connection between autoimmunity and ALS/FTD, and explores the possibility that C9orf72 and other ALS/FTD genes may have a “dual effect” on both neuronal and myeloid cell function that could explain a shared propensity for altered systemic immunity and neurodegeneration.

肌萎缩性侧索硬化症（ALS）是一种退行性疾病，其特征在于运动神经元缺失，并表现出与额颞痴呆（FTD）的临床，病理和遗传重叠。活化的小胶质细胞是ALS / FTD病理学的普遍特征。然而，它们在疾病发病机理中的作用仍不完全清楚。最近发现，在ALS / FTD中最常突变的9号染色体上的ORF 72（C9orf72）在髓样细胞中具有重要作用，这开启了改变的小胶质细胞功能在疾病中发挥积极作用的可能性。这篇评论重点介绍了小胶质细胞对ALS / FTD发病机制的贡献，并讨论了自身免疫与ALS / FTD之间的联系，