Zoltowska Katarzyna Marta, Das Utpal, Lismont Sam, Enzlein Thomas, Maesako Masato, Houser Mei Cq, Franco María Luisa, Özcan Burcu, Moreira Diana Gomes, Karachentsev Dmitry, Becker Ann, Hopf Carsten, Vilar Marçal, Berezovska Oksana, Mobley William, Chávez-Gutiérrez Lucía
VIB-KU Leuven Center for Brain & Disease Research, VIB, Leuven, Belgium.
Department of Neurosciences, University of California San Diego, La Jolla, CA, United States of America.
bioRxiv. 2024 Apr 23:2023.08.02.551596. doi: 10.1101/2023.08.02.551596.
Amyloid β (Aβ) peptides accumulating in the brain are proposed to trigger Alzheimer's disease (AD). However, molecular cascades underlying their toxicity are poorly defined. Here, we explored a novel hypothesis for Aβ42 toxicity that arises from its proven affinity for γ-secretases. We hypothesized that the reported increases in Aβ42, particularly in the endolysosomal compartment, promote the establishment of a product feedback inhibitory mechanism on γ-secretases, and thereby impair downstream signaling events. We show that human Aβ42 peptides, but neither murine Aβ42 nor human Aβ17-42 (p3), inhibit γ-secretases and trigger accumulation of unprocessed substrates in neurons, including C-terminal fragments (CTFs) of APP, p75 and pan-cadherin. Moreover, Aβ42 treatment dysregulated cellular -homeostasis, as shown by the induction of p75-dependent neuronal death in two distinct cellular systems. Our findings raise the possibility that pathological elevations in Aβ42 contribute to cellular toxicity via the γ-secretase inhibition, and provide a novel conceptual framework to address Aβ toxicity in the context of γ-secretase-dependent homeostatic signaling.
大脑中积累的淀粉样β(Aβ)肽被认为会引发阿尔茨海默病(AD)。然而,其毒性背后的分子级联反应却知之甚少。在此,我们探讨了一种关于Aβ42毒性的新假说,该假说源于其已被证实的对γ-分泌酶的亲和力。我们假设,报道中Aβ42的增加,尤其是在内溶酶体区室中的增加,会促进对γ-分泌酶建立一种产物反馈抑制机制,从而损害下游信号事件。我们发现,人源Aβ42肽,但不是鼠源Aβ42也不是人源Aβ17-42(p3),会抑制γ-分泌酶并引发神经元中未加工底物的积累,包括APP的C端片段(CTFs)、p75和泛钙黏蛋白。此外,Aβ42处理会使细胞内稳态失调,这在两个不同的细胞系统中p75依赖的神经元死亡诱导中得到了体现。我们的研究结果提出了一种可能性,即Aβ42的病理性升高通过γ-分泌酶抑制导致细胞毒性,并提供了一个新的概念框架,以在γ-分泌酶依赖的稳态信号背景下解决Aβ毒性问题。