The Conserved Oligomeric Golgi (COG) complex, a multi-subunit vesicle tethering complex of the CATCHR (Complexes Associated with Tethering Containing Helical Rods) family, controls several aspects of cellular homeostasis by orchestrating retrograde vesicle traffic within the Golgi. The COG complex interacts with all key players regulating intra-Golgi trafficking, namely SNAREs, SNARE-interacting proteins, Rabs, coiled-coil tethers, and vesicular coats. In cells, COG deficiencies result in the accumulation of non-tethered COG-complex dependent (CCD) vesicles, dramatic morphological and functional abnormalities of the Golgi and endosomes, severe defects in N- and O- glycosylation, Golgi retrograde trafficking, sorting and protein secretion. In humans, COG mutations lead to severe multi-systemic diseases known as COG-Congenital Disorders of Glycosylation (COG-CDG). In this report, we review the current knowledge of the COG complex and analyze COG-related trafficking and glycosylation defects in COG-CDG patients.

保守寡聚高尔基体（COG）复合物是CATCHR（与包含螺旋杆的束缚相关联的复合物）家族的多亚基囊泡束缚复合物，通过协调高尔基体内的逆行囊泡运输来控制细胞稳态的多个方面。COG复合物与所有调节高尔基体内运输的关键因素相互作用，即SNARE，与SNARE相互作用的蛋白，Rabs，卷曲螺旋系绳和囊泡被膜。在细胞中，COG缺乏会导致非束缚的COG复杂依赖（CCD）囊泡堆积，高尔基体和内体的剧烈形态和功能异常，N-和O-糖基化严重缺陷，高尔基逆行运输，分选和蛋白质分泌。在人类中 COG突变会导致严重的多系统疾病，称为COG-先天性糖基化障碍（COG-CDG）。在本报告中，我们回顾了COG复合物的当前知识，并分析了COG-CDG患者中COG相关的运输和糖基化缺陷。