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Trends Cell Biol. 2014 Jan;24(1):9-18. doi: 10.1016/j.tcb.2013.08.005. Epub 2013 Sep 26.

Exiting the ER: what we know and what we don't.

Author information

1
Telethon Institute of Genetics and Medicine, Via Pietro Castellino 111, Naples 80131, Italy.
2
Telethon Institute of Genetics and Medicine, Via Pietro Castellino 111, Naples 80131, Italy. Electronic address: dematteis@tigem.it.

Abstract

The vast majority of proteins that are transported to different cellular compartments and secreted from the cell require coat protein complex II (COPII) for export from the endoplasmic reticulum (ER). Many of the molecular mechanisms underlying COPII assembly are understood in great detail, but it is becoming increasingly evident that this basic machinery is insufficient to account for diverse aspects of protein export from the ER that are observed in vivo. Here we review recent data that have furthered our mechanistic understanding of COPII assembly and, in particular, how genetic diseases associated with the early secretory pathway have added fundamental insights into the regulation of ER-derived carrier formation. We also highlight some unresolved issues that future work should address to better understand the physiology of COPII-mediated transport.

KEYWORDS:

COPII coat; ER export; ER-derived carriers; genetic disease of membrane trafficking

PMID:
24076263
DOI:
10.1016/j.tcb.2013.08.005
[Indexed for MEDLINE]

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