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Generation of Plasmid Vectors Expressing FLAG-tagged Proteins Under the Regulation of Human Elongation Factor-1α Promoter Using Gibson Assembly
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Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.

Lisa M Sharkey1,2, Nathaniel Safren1, Amit S Pithadia1

  • 1Department of Neurology, University of Michigan, Ann Arbor, MI 48109-2200.

Proceedings of the National Academy of Sciences of the United States of America
|October 19, 2018
PubMed
Summary

The protein UBQLN2 self-assembles into aggregates, a process linked to neurodegenerative diseases like ALS. Pathogenic mutations disrupt this self-assembly, increasing neurotoxicity and disease risk.

Keywords:
ALSFTDUBQLN2liquid–liquid phase separationmembraneless organelle

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Area of Science:

  • Molecular Biology
  • Neuroscience
  • Protein Biochemistry

Background:

  • UBQLN2 is involved in protein quality control and linked to neurodegenerative diseases.
  • Mutations in UBQLN2 cause X-linked amyotrophic lateral sclerosis/frontotemporal dementia.
  • Wild-type UBQLN2 aggregates in age-related neurodegenerative diseases.

Purpose of the Study:

  • To investigate the self-assembly properties of UBQLN2.
  • To understand how UBQLN2 mutations affect its aggregation and neurotoxicity.
  • To elucidate the link between UBQLN2's function and its aggregation propensity.

Main Methods:

  • In vitro protein analysis
  • Longitudinal fluorescence imaging
  • Cellular, neuronal, and transgenic mouse models

Main Results:

  • UBQLN2 intrinsically self-assembles into liquid-like droplets and amyloid aggregates.
  • UBQLN2 self-assembly and solubility are modulated by its ubiquitin-like and ubiquitin-associated domains.
  • A pathogenic UBQLN2 mutation impairs droplet dynamics and promotes neurotoxic amyloid aggregation.

Conclusions:

  • UBQLN2's propensity to self-assemble is critical in neurodegenerative diseases.
  • Dysfunctional UBQLN2 aggregation contributes to neurotoxicity.
  • Understanding UBQLN2 self-assembly pathways is key for therapeutic strategies.