Department of Research Service, Omaha Western Iowa Health Care System, VA Service, Omaha, NE, USA.
Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
Hepatol Commun. 2022 Feb;6(2):374-388. doi: 10.1002/hep4.1811. Epub 2021 Sep 7.
Previous work from our laboratories has identified multiple defects in endocytosis, protein trafficking, and secretion, along with altered Golgi function after alcohol administration. Manifestation of alcohol-associated liver disease (ALD) is associated with an aberrant function of several hepatic proteins, including asialoglycoprotein receptor (ASGP-R), their atypical distribution at the plasma membrane (PM), and secretion of their abnormally glycosylated forms into the bloodstream, but trafficking mechanism is unknown. Here we report that a small GTPase, Rab3D, known to be involved in exocytosis, secretion, and vesicle trafficking, shows ethanol (EtOH)-impaired function, which plays an important role in Golgi disorganization. We used multiple approaches and cellular/animal models of ALD, along with Rab3D knockout (KO) mice and human tissue from patients with ALD. We found that Rab3D resides primarily in trans- and cis-faces of Golgi; however, EtOH treatment results in Rab3D redistribution from trans-Golgi to cis-medial-Golgi. Cells lacking Rab3D demonstrate enlargement of Golgi, especially its distal compartments. We identified that Rab3D is required for coat protein I (COPI) vesiculation in Golgi, and conversely, COPI is critical for intra-Golgi distribution of Rab3D. Rab3D/COPI association was altered not only in the liver of patients with ALD but also in the donors consuming alcohol without steatosis. In Rab3D KO mice, hepatocytes experience endoplasmic reticulum (ER) stress, and EtOH administration activates apoptosis. Notably, in these cells, ASGP-R, despite incomplete glycosylation, can still reach cell surface through ER-PM junctions. This mimics the effects seen with EtOH-induced liver injury. Conclusion: We revealed that down-regulation of Rab3D contributes significantly to EtOH-induced Golgi disorganization, and abnormally glycosylated ASGP-R is excreted through ER-PM connections, bypassing canonical (ER→Golgi→PM) anterograde transportation. This suggests that ER-PM sites may be a therapeutic target for ALD.
先前我们实验室的工作已经确定,在酒精给药后,会出现多个内吞作用、蛋白质运输和分泌缺陷以及高尔基体功能改变。酒精相关性肝病 (ALD) 的表现与几种肝蛋白的异常功能有关,包括去唾液酸糖蛋白受体 (ASGP-R),它们在质膜 (PM) 上的异常分布,以及它们异常糖基化形式分泌到血液中,但运输机制尚不清楚。在这里,我们报告说,一种小 GTPase,Rab3D,已知参与胞吐作用、分泌和囊泡运输,其功能在乙醇 (EtOH) 作用下受损,在高尔基体解体中起着重要作用。我们使用了多种方法和 ALD 的细胞/动物模型,以及 Rab3D 敲除 (KO) 小鼠和来自 ALD 患者的人类组织。我们发现 Rab3D 主要存在于高尔基体的顺面和反面;然而,EtOH 处理导致 Rab3D 从反式高尔基体重新分布到顺式中间高尔基体。缺乏 Rab3D 的细胞表现出高尔基体增大,特别是其远端隔室。我们发现 Rab3D 是高尔基体中衣壳蛋白 I (COPI) 小泡形成所必需的,反之,COPI 对 Rab3D 在高尔基体中的分布至关重要。不仅在 ALD 患者的肝脏中,而且在没有脂肪变性的饮酒供体中,Rab3D/COPI 结合也发生改变。在 Rab3D KO 小鼠中,肝细胞经历内质网 (ER) 应激,EtOH 给药激活细胞凋亡。值得注意的是,在这些细胞中,尽管 ASGP-R 不完全糖基化,但仍可以通过 ER-PM 连接到达细胞表面。这模拟了 EtOH 诱导的肝损伤所观察到的效果。结论:我们揭示了 Rab3D 的下调显著导致 EtOH 诱导的高尔基体解体,并且异常糖基化的 ASGP-R 通过 ER-PM 连接排出,绕过了典型的 (ER→Golgi→PM) 顺行运输。这表明 ER-PM 部位可能是 ALD 的治疗靶点。