Nuclear pore complex and nucleocytoplasmic transport disruption in neurodegeneration.
ALS
ESCRT
FTD
neurodegeneration
nuclear pore complex
nucleocytoplasmic transport
nucleoporins
Journal
FEBS letters
ISSN: 1873-3468
Titre abrégé: FEBS Lett
Pays: England
ID NLM: 0155157
Informations de publication
Date de publication:
10 2023
10 2023
Historique:
revised:
29
07
2023
received:
25
06
2023
accepted:
24
08
2023
pmc-release:
01
10
2024
medline:
30
10
2023
pubmed:
2
9
2023
entrez:
1
9
2023
Statut:
ppublish
Résumé
Nuclear pore complexes (NPCs) play a critical role in maintaining the equilibrium between the nucleus and cytoplasm, enabling bidirectional transport across the nuclear envelope, and are essential for proper nuclear organization and gene regulation. Perturbations in the regulatory mechanisms governing NPCs and nuclear envelope homeostasis have been implicated in the pathogenesis of several neurodegenerative diseases. The ESCRT-III pathway emerges as a critical player in the surveillance and preservation of well-assembled, functional NPCs, as well as nuclear envelope sealing. Recent studies have provided insights into the involvement of nuclear ESCRT-III in the selective reduction of specific nucleoporins associated with neurodegenerative pathologies. Thus, maintaining quality control of the nuclear envelope and NPCs represents a pivotal element in the pathological cascade leading to neurodegenerative diseases. This review describes the constituents of the nuclear-cytoplasmic transport machinery, encompassing the nuclear envelope, NPC, and ESCRT proteins, and how their structural and functional alterations contribute to the development of neurodegenerative diseases.
Identifiants
pubmed: 37657945
doi: 10.1002/1873-3468.14729
pmc: PMC10612469
mid: NIHMS1936872
doi:
Substances chimiques
Nuclear Pore Complex Proteins
0
Endosomal Sorting Complexes Required for Transport
0
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
2546-2566Subventions
Organisme : NINDS NIH HHS
ID : R00 NS123242
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS132836
Pays : United States
Informations de copyright
© 2023 Federation of European Biochemical Societies.
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