Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases

Most proteins destined for the extracellular space or various intracellular compartments must traverse the intracellular secretory pathway. The first step is the recruitment and transport of cargoes from the endoplasmic reticulum (ER) lumen to the Golgi apparatus by coat protein complex II (COPII),...

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Main Authors: Vi T. Tang, David Ginsburg
Format: Article
Language:English
Published: American Society for Clinical Investigation 2023-01-01
Series:The Journal of Clinical Investigation
Online Access:https://doi.org/10.1172/JCI163838
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author Vi T. Tang
David Ginsburg
author_facet Vi T. Tang
David Ginsburg
author_sort Vi T. Tang
collection DOAJ
description Most proteins destined for the extracellular space or various intracellular compartments must traverse the intracellular secretory pathway. The first step is the recruitment and transport of cargoes from the endoplasmic reticulum (ER) lumen to the Golgi apparatus by coat protein complex II (COPII), consisting of five core proteins. Additional ER transmembrane proteins that aid cargo recruitment are referred to as cargo receptors. Gene duplication events have resulted in multiple COPII paralogs present in the mammalian genome. Here, we review the functions of each COPII protein, human disorders associated with each paralog, and evidence for functional conservation between paralogs. We also provide a summary of current knowledge regarding two prototypical cargo receptors in mammals, LMAN1 and SURF4, and their roles in human health and disease.
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spelling doaj.art-ee1bd5cbd9e740bca717094050de240e2023-11-07T16:19:42ZengAmerican Society for Clinical InvestigationThe Journal of Clinical Investigation1558-82382023-01-011331Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseasesVi T. TangDavid GinsburgMost proteins destined for the extracellular space or various intracellular compartments must traverse the intracellular secretory pathway. The first step is the recruitment and transport of cargoes from the endoplasmic reticulum (ER) lumen to the Golgi apparatus by coat protein complex II (COPII), consisting of five core proteins. Additional ER transmembrane proteins that aid cargo recruitment are referred to as cargo receptors. Gene duplication events have resulted in multiple COPII paralogs present in the mammalian genome. Here, we review the functions of each COPII protein, human disorders associated with each paralog, and evidence for functional conservation between paralogs. We also provide a summary of current knowledge regarding two prototypical cargo receptors in mammals, LMAN1 and SURF4, and their roles in human health and disease.https://doi.org/10.1172/JCI163838
spellingShingle Vi T. Tang
David Ginsburg
Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
The Journal of Clinical Investigation
title Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
title_full Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
title_fullStr Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
title_full_unstemmed Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
title_short Cargo selection in endoplasmic reticulum–to–Golgi transport and relevant diseases
title_sort cargo selection in endoplasmic reticulum to golgi transport and relevant diseases
url https://doi.org/10.1172/JCI163838
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