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Review
. 2023 Oct;597(20):2534-2545.
doi: 10.1002/1873-3468.14725. Epub 2023 Aug 30.

Coordinating nucleoporin condensation and nuclear pore complex assembly

E F Elsiena Kuiper  1 , Sarah M Prophet  1 , Christian Schlieker  1   2
Affiliations
Free article
Review

Coordinating nucleoporin condensation and nuclear pore complex assembly

E F Elsiena Kuiper et al. FEBS Lett. 2023 Oct.
Free article

Abstract

The nuclear pore complex (NPC) is among the most elaborate protein complexes in eukaryotes. While ribosomes and proteasomes are known to require dedicated assembly machinery, our understanding of NPC assembly is at a relatively early stage. Defects in NPC assembly or homeostasis are tied to movement disorders, including dystonia and amyotrophic lateral sclerosis (ALS), as well as aging, requiring a better understanding of these processes to enable therapeutic intervention. Here, we discuss recent progress in the understanding of NPC assembly and highlight how related defects in human disorders can shed light on NPC biogenesis. We propose that the condensation of phenylalanine-glycine repeat nucleoporins needs to be carefully controlled during NPC assembly to prevent aberrant condensation, aggregation, or amyloid formation.

Keywords: condensation; fusogen; membrane fusion; molecular chaperones; nuclear envelope; nuclear pore complex; phase separation.

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