1. Golgi inCOGnito: From vesicle tethering to human disease.
- Author
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D'Souza Z, Taher FS, and Lupashin VV
- Subjects
- Adaptor Proteins, Vesicular Transport genetics, Animals, Biological Transport, Congenital Disorders of Glycosylation genetics, Congenital Disorders of Glycosylation pathology, Glycosylation, Golgi Apparatus genetics, Golgi Apparatus pathology, Humans, Multiprotein Complexes genetics, Mutation, Protein Interaction Maps, Protein Subunits genetics, Protein Subunits metabolism, Adaptor Proteins, Vesicular Transport metabolism, Congenital Disorders of Glycosylation metabolism, Golgi Apparatus metabolism, Multiprotein Complexes metabolism
- Abstract
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., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier B.V. All rights reserved.)
- Published
- 2020
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