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以α-氨基-3-羟基-4异恶唑-丙酸(AMPA)受体介导的Ca~(2+)通透性增加,Ca~(2+)流入比例增大而导致神经细胞死亡的理论已经明确。AMPA受体的谷氨酸受体2(GluR2)亚基的Q/R部位编辑率低下是散发性肌萎缩性侧索硬化(ALS)运动神经元死亡的主要原因。变异的铜-锌超氧化物歧化酶(SOD1)相关的家族性ALS(ALS1)是以AMPA受体含GluR2亚基的比例减少为基础;以延髓脊髓性肌萎缩症(SMBA)为代表运动神经元的死亡,不是AMPA受体所介导。ALS GluR2 Q/R部位核糖核酸(RNA)编辑的恢复可以考虑成为ALS治疗的特异方法,而AMPA受体拮抗剂可以防止ALS1运动神经元的变性。
The increase of Ca 2+ permeability mediated by α-amino-3-hydroxy-4-isoxazole-propionic acid (AMPA) receptor leads to the increase of Ca 2+ influx, resulting in the death of nerve cells The theory has been clear. Poor editing of the Q / R site of the glutamate receptor 2 (GluR2) subunit of the AMPA receptor is a major cause of death from sporadic amyotrophic lateral sclerosis (ALS) motor neurons. Variant copper-zinc superoxide dismutase (SOD1) -related familial ALS (ALS1) is based on AMPA receptor containing GluR2 subunit reduction was based; with medullary spinal muscular atrophy (SMBA) as the representative motor nerve Meta-death is not mediated by AMPA receptors. Recovery of ALS GluR2 Q / R RNA editing can be considered as a specific treatment for ALS, whereas AMPA receptor antagonists prevent denaturation of ALS1 motor neurons.