title: Border Safety: Quality Control at the Nuclear Envelope.
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.
authors.affiliation: Yale School of Medicine, New Haven, CT, USA.
authors.forename: Brant M
authors.initials: BM
authors.lastname: Webster
authors.affiliation: Yale School of Medicine, New Haven, CT, USA. Electronic address: patrick.lusk@yale.edu.
authors.forename: C Patrick
authors.initials: CP
authors.lastname: Lusk
mesh_terms: D021581:Active Transport, Cell Nucleus; D000818:Animals; D060746:Endoplasmic Reticulum-Associated Degradation; D056827:Endosomal Sorting Complexes Required for Transport / Q000502:physiology; D006801:Humans; D009685:Nuclear Envelope / Q000502:physiology*; D009687:Nuclear Proteins / Q000378:metabolism
chemical_list: D056827:Endosomal Sorting Complexes Required for Transport; D009687:Nuclear Proteins
keywords: ERAD; ESCRT; compartmentalization; inner nuclear membrane; nuclear envelope; nuclear pore complex; quality control