Coordinating nucleoporin condensation and nuclear pore complex assembly

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.

Document Details

Document Type
Pub Defense Publication
Publication Date
Aug 30, 2023
Source ID
10.1002/1873-3468.14725

Entities

People

  • Christian Schlieker
  • Els F E Kuiper
  • Sarah Prophet

Organizations

  • Dutch Research Council
  • Dystonia Medical Research Foundation
  • European Molecular Biology Organization
  • United States Department of Defense
  • Yale School of Medicine
  • Yale University

Tags

Readers

  • Child and Adolescent Substance Abuse Science in Autism Spectrum Disorders.
  • Molecular Genetics
  • Molecular and Cellular Biology