School of Life Sciences, Faculty of Science, The Chinese University of Hong Kong, Hong Kong SAR, China.
Laboratory of Neurodegenerative Diseases, School of Biomedical Sciences, LKS Faculty of Medicine, and State Key Laboratory of Brain and Cognitive Sciences, The University of Hong Kong, Hong Kong SAR, China.
Cell Mol Life Sci. 2024 Jul 30;81(1):323. doi: 10.1007/s00018-024-05351-8.
Autophagy is a highly conserved catabolic mechanism by which unnecessary or dysfunctional cellular components are removed. The dysregulation of autophagy has been implicated in various neurodegenerative diseases, including Alzheimer's disease (AD). Understanding the molecular mechanism(s)/molecules that influence autophagy may provide important insights into developing therapeutic strategies against AD and other neurodegenerative disorders. Engulfment adaptor phosphotyrosine-binding domain-containing protein 1 (GULP1) is an adaptor that interacts with amyloid precursor protein (APP) to promote amyloid-β peptide production via an unidentified mechanism. Emerging evidence suggests that GULP1 has a role in autophagy. Here, we show that GULP1 is involved in autophagy through an interaction with autophagy-related 14 (ATG14), which is a regulator of autophagosome formation. GULP1 potentiated the stimulatory effect of ATG14 on autophagy by modulating class III phosphatidylinositol 3-kinase complex 1 (PI3KC3-C1) activity. The effect of GULP1 is attenuated by a GULP1 mutation (GULP1m) that disrupts the GULP1-ATG14 interaction. Conversely, PI3KC3-C1 activity is enhanced in cells expressing APP but not in those expressing an APP mutant that does not bind GULP1, which suggests a role of GULP1-APP in regulating PI3KC3-C1 activity. Notably, GULP1 facilitates the targeting of ATG14 to the endoplasmic reticulum (ER). Moreover, the levels of both ATG14 and APP are elevated in the autophagic vacuoles (AVs) of cells expressing GULP1, but not in those expressing GULP1m. APP processing is markedly enhanced in cells co-expressing GULP1 and ATG14. Hence, GULP1 alters APP processing by promoting the entry of APP into AVs. In summary, we unveil a novel role of GULP1 in enhancing the targeting of ATG14 to the ER to stimulate autophagy and, consequently, APP processing.
自噬是一种高度保守的分解代谢机制,通过该机制可以清除不必要或功能失调的细胞成分。自噬的失调与多种神经退行性疾病有关,包括阿尔茨海默病(AD)。了解影响自噬的分子机制/分子可能为开发针对 AD 和其他神经退行性疾病的治疗策略提供重要的见解。吞噬衔接蛋白磷酸酪氨酸结合域包含蛋白 1(GULP1)是一种衔接蛋白,通过一种未知的机制与淀粉样前体蛋白(APP)相互作用,促进淀粉样β肽的产生。新出现的证据表明,GULP1 在自噬中起作用。在这里,我们通过与自噬相关 14 号(ATG14)的相互作用表明 GULP1 参与自噬,ATG14 是自噬体形成的调节剂。GULP1 通过调节 III 类磷酸肌醇 3-激酶复合物 1(PI3KC3-C1)活性,增强了 ATG14 对自噬的刺激作用。GULP1 突变(GULP1m)破坏了 GULP1-ATG14 相互作用,从而减弱了 GULP1 的作用。相反,在表达 APP 但不表达不与 GULP1 结合的 APP 突变体的细胞中,PI3KC3-C1 活性增强,这表明 GULP1-APP 在调节 PI3KC3-C1 活性中起作用。值得注意的是,GULP1 促进 ATG14 靶向内质网(ER)。此外,在表达 GULP1 的细胞的自噬小泡(AVs)中,ATG14 和 APP 的水平均升高,但在表达 GULP1m 的细胞中则不然。在共表达 GULP1 和 ATG14 的细胞中,APP 处理明显增强。因此,GULP1 通过促进 APP 进入 AVs 来改变 APP 处理。总之,我们揭示了 GULP1 在促进 APP 进入 AVs 以刺激自噬,进而促进 APP 处理方面的新作用。