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How Do Yeast Cells Contend with Prions?

Reed B Wickner1, Herman K Edskes1, Moonil Son1

  • 1Laboratory of Biochemistry and Genetics, National Institute of Diabetes Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-0830, USA.

International Journal of Molecular Sciences
|July 9, 2020
PubMed

Insights

Yeast possess eight anti-prion systems that prevent, cure, or limit pathogenic amyloid prions. Understanding these systems may help develop human prion disease treatments.

Area of Science:

  • Molecular Biology
  • Biochemistry
  • Genetics

Background:

  • Prions are infectious proteins forming amyloid filaments, implicated in human and yeast diseases.
  • Yeast models offer insights into prion biology and potential therapeutic strategies.

Purpose of the Study:

  • To identify and characterize anti-prion systems in Saccharomyces cerevisiae.
  • To elucidate the mechanisms employed by yeast to combat pathogenic prions.

Main Methods:

  • Genetic screens to identify anti-prion genes.
  • Biochemical assays to study protein folding and filament formation.
  • Cellular imaging to track prion propagation and segregation.

Main Results:

  • Eight distinct anti-prion systems identified in yeast.
  • Mechanisms include blocking prion generation, curing existing prions, and limiting pathogenicity.
  • Specific genes (e.g., Hsp104, Btn2, Sis1) and pathways (e.g., inositol polyphosphate regulation) are involved.

Conclusions:

  • Yeast possess robust defense mechanisms against amyloid prions.
  • Elucidating these systems provides a foundation for understanding and potentially treating human prion diseases.
Keywords:
Btn2Cur1Hsp104Lug1Siw14SsbUpfamyloidanti-prionprion

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