Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA.
Department of Neurology, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China.
Sci Adv. 2021 Apr 16;7(16). doi: 10.1126/sciadv.abe4499. Print 2021 Apr.
Netrin-1, a family member of laminin-related secreted proteins, mediates axon guidance and cell migration during neural development. T835M mutation in netrin receptor UNC5C predisposes to the late-onset Alzheimer's disease (AD) and increases neuronal cell death. However, it remains unclear how this receptor is molecularly regulated in AD. Here, we show that δ-secretase selectively cleaves UNC5C and escalates its proapoptotic activity, facilitating neurodegeneration in AD. Netrin deficiency activates δ-secretase that specifically cuts UNC5C at N467 and N547 residues and enhances subsequent caspase-3 activation, additively augmenting neuronal cell death. Blockade of δ-secretase cleavage of UNC5C diminishes T835M mutant's proapoptotic activity. Viral expression of δ-secretase-truncated UNC5C fragments into APP/PS1 mice strongly accelerates AD pathologies, impairing learning and memory. Conversely, deletion of UNC5C from netrin-1-depleted mice attenuates AD pathologies and rescues cognitive disorders. Hence, δ-secretase truncates UNC5C and elevates its neurotoxicity, contributing to AD pathogenesis.
轴突导向和细胞迁移的神经营养因子 UNC5C 受体
神经导向因子 UNC5C 是一种层粘连蛋白相关分泌蛋白家族成员,在神经发育过程中介导轴突导向和细胞迁移。UNC5C 中的 T835M 突变易患晚发性阿尔茨海默病(AD),并增加神经元细胞死亡。然而,目前尚不清楚该受体在 AD 中的分子调控机制。
在这里,我们发现 δ 分泌酶选择性地切割 UNC5C,增加其促凋亡活性,促进 AD 中的神经退行性变。无 netrin 时激活 δ 分泌酶,特异性切割 UNC5C 的 N467 和 N547 残基,增强随后的 caspase-3 激活,从而增加神经元细胞死亡。阻断 δ 分泌酶切割 UNC5C 可降低 T835M 突变体的促凋亡活性。APP/PS1 小鼠中表达 δ 分泌酶截断的 UNC5C 片段可强烈加速 AD 病理,损害学习和记忆。相反,从 netrin-1 耗竭的小鼠中删除 UNC5C 可减轻 AD 病理并挽救认知障碍。因此,δ 分泌酶截断 UNC5C 并增加其神经毒性,导致 AD 发病机制。