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),...
Main Authors: | , |
---|---|
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 |
_version_ | 1797634541255393280 |
---|---|
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. |
first_indexed | 2024-03-11T12:09:16Z |
format | Article |
id | doaj.art-ee1bd5cbd9e740bca717094050de240e |
institution | Directory Open Access Journal |
issn | 1558-8238 |
language | English |
last_indexed | 2024-03-11T12:09:16Z |
publishDate | 2023-01-01 |
publisher | American Society for Clinical Investigation |
record_format | Article |
series | The Journal of Clinical Investigation |
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 |
work_keys_str_mv | AT vittang cargoselectioninendoplasmicreticulumtogolgitransportandrelevantdiseases AT davidginsburg cargoselectioninendoplasmicreticulumtogolgitransportandrelevantdiseases |