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Trends Cell Biol. 2016 Jan;26(1):29-39. doi: 10.1016/j.tcb.2015.08.002. Epub 2015 Oct 1.

Border Safety: Quality Control at the Nuclear Envelope.

Author information

1
Yale School of Medicine, New Haven, CT, USA.
2
Yale School of Medicine, New Haven, CT, USA. Electronic address: patrick.lusk@yale.edu.

Abstract

The unique biochemical identity of the nuclear envelope confers its capacity to establish a barrier that protects the nuclear compartment and directly contributes to nuclear function. Recent work uncovered quality control mechanisms employing the endosomal sorting complexes required for transport (ESCRT) machinery and a new arm of endoplasmic reticulum-associated protein degradation (ERAD) to counteract the unfolding, damage, or misassembly of nuclear envelope proteins and ensure the integrity of the nuclear envelope membranes. Moreover, cells have the capacity to recognize and triage defective nuclear pore complexes to prevent their inheritance and preserve the longevity of progeny. These mechanisms serve to highlight the diverse strategies used by cells to maintain nuclear compartmentalization; we suggest they mitigate the progression and severity of diseases associated with nuclear envelope malfunction such as the laminopathies.

KEYWORDS:

ERAD; ESCRT; compartmentalization; inner nuclear membrane; nuclear envelope; nuclear pore complex; quality control

PMID:
26437591
PMCID:
PMC4698192
DOI:
10.1016/j.tcb.2015.08.002
[Indexed for MEDLINE]
Free PMC Article

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