Immune-mediated Mechanisms in the Pathoprogression of Amyotrophic Lateral Sclerosis

摘要: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with selective loss of upper and lower motor neurons. At sites neuron injury, neuroinflammation prominent pathological finding characterized by microglial activation, astrogliosis, infiltration monocytes T-cells. Both innate adaptive immune responses actively influence progression in animal models ALS patients, promote neuroprotection or neurotoxicity at different stages disease. The early reaction to signals from injured neurons rescue repair damaged tissue. As accelerates, shift occurs beneficial (involving M2 microglia regulatory T-cells) deleterious M1 Th1 cells). In this review, we underscore the importance immune-mediated mechanisms pathogenesis discuss alterations distinct phenotypes cells better understand dynamic changes that occur within system over course disease, will be able develop effective therapeutic regimens ALS.