Khakurel Amrita, Pokrovskaya Irina, Lupashin Vladimir V
University of Arkansas for Medical Sciences, Department of Physiology and Cell Biology, Little Rock, Arkansas, US.
bioRxiv. 2024 Oct 14:2024.10.07.617053. doi: 10.1101/2024.10.07.617053.
The GARP complex is an evolutionarily conserved protein complex proposed to tether endosome-derived vesicles at the trans-Golgi network. While prolonged depletion of GARP leads to severe trafficking and glycosylation defects, the primary defects linked to GARP dysfunction remain unclear. In this study, we utilized the mAID degron strategy to achieve rapid degradation of VPS54 in human cells, acutely disrupting GARP function. This resulted in the partial mislocalization and degradation of a subset of Golgi-resident proteins, including TGN46, ATP7A, TMEM87A, CPD, C1GALT1, and GS15. Enzyme recycling defects led to the early onset of O-glycosylation abnormalities. Additionally, while the secretion of fibronectin and cathepsin D was altered, mannose-6-phosphate receptors were largely unaffected. Partial displacement of COPI, AP1, and GGA coats caused a significant accumulation of vesicle-like structures and large vacuoles. Electron microscopy detection of GARP-dependent vesicles, along with the identification of specific cargo proteins, provides direct experimental evidence of GARP's role as a vesicular tether. We conclude that the primary defects of GARP dysfunction involve vesicular coat mislocalization, accumulation of GARP-dependent vesicles, degradation and mislocalization of specific Golgi proteins, and O-glycosylation defects.
GARP复合物是一种进化上保守的蛋白质复合物,被认为可将内体来源的囊泡拴系在反式高尔基体网络上。虽然长期耗尽GARP会导致严重的运输和糖基化缺陷,但与GARP功能障碍相关的主要缺陷仍不清楚。在本研究中,我们利用mAID降解子策略在人类细胞中实现VPS54的快速降解,从而急性破坏GARP功能。这导致了一部分高尔基体驻留蛋白的部分错误定位和降解,包括TGN46、ATP7A、TMEM87A、CPD、C1GALT1和GS15。酶循环缺陷导致O-糖基化异常提前出现。此外,虽然纤连蛋白和组织蛋白酶D的分泌发生了改变,但甘露糖-6-磷酸受体基本未受影响。COPI、AP1和GGA衣被的部分移位导致囊泡样结构和大液泡大量积累。通过电子显微镜检测GARP依赖性囊泡以及鉴定特定的货物蛋白,为GARP作为囊泡拴系蛋白的作用提供了直接的实验证据。我们得出结论,GARP功能障碍的主要缺陷包括囊泡衣被的错误定位、GARP依赖性囊泡的积累、特定高尔基体蛋白的降解和错误定位以及O-糖基化缺陷。