Shanghai Key Laboratory of Signaling and Diseases Research, School of Life Science and Technology, The First Rehabilitation Hospital of Shanghai, Tongji University, Shanghai, China.
Mathey College, Princeton University, Princeton, New Jersey, USA.
J Cell Physiol. 2023 Jul;238(7):1567-1579. doi: 10.1002/jcp.31035. Epub 2023 May 14.
The amyloid-β (Aβ) peptide, produced from amyloid precursor protein (APP) by β and γ-secretases, has been implicated in the etiology of Alzheimer's disease (AD). However, the precise intracellular trafficking pathway of APP and its subcellular locations to produce Aβ have remained unclear. To address these issues, we established fly AD models that recapitulated multiple AD-like symptoms by expressing human APP in the Drosophila nerve system. The ESCRT (endosomal sorting complexes required for transport) machinery regulates the sorting and trafficking of endocytosed proteins, yet its role in AD pathogenesis has not been explored in vivo. We found that knockdown of distinct ESCRT components ameliorated APP-induced morphological and behavioral defects, including impaired wing expansion, eye degeneration, dopamine neuron loss, locomotor disability, lifespan shortening, and cognitive deficits. Mechanistically, we showed that impaired ESCRT impeded APP's intracellular transportation from early endosomes to late endosomes, resulting in reduced Aβ production and amyloid deposit load. These data suggest that APP undergoes ESCRT-mediated endocytic trafficking, and Aβ is generated mainly in late endosomes. Our data provide the first in vivo evidence to support a physiological role of ESCRT in AD pathogenesis, suggesting that interfering with ESCRT machinery might be an alternative therapeutic strategy for AD.
淀粉样蛋白-β(Aβ)肽由β和γ-分泌酶从淀粉样前体蛋白(APP)产生,与阿尔茨海默病(AD)的病因有关。然而,APP 的确切细胞内转运途径及其产生 Aβ的亚细胞位置仍不清楚。为了解决这些问题,我们通过在果蝇神经系统中表达人 APP 建立了模拟多种 AD 样症状的果蝇 AD 模型。ESCRT(内体分选复合物必需的运输)机制调节内吞蛋白的分拣和运输,但它在 AD 发病机制中的作用尚未在体内进行探索。我们发现,敲低不同的 ESCRT 成分可改善 APP 诱导的形态和行为缺陷,包括翅膀扩张受损、眼睛退化、多巴胺神经元丧失、运动障碍、寿命缩短和认知缺陷。从机制上讲,我们表明受损的 ESCRT 阻碍了 APP 从早期内体到晚期内体的细胞内运输,导致 Aβ产生减少和淀粉样沉积负荷降低。这些数据表明 APP 经历了 ESCRT 介导的内吞运输,Aβ主要在晚期内体中产生。我们的数据提供了体内证据支持 ESCRT 在 AD 发病机制中的生理作用,表明干扰 ESCRT 机制可能是 AD 的一种替代治疗策略。