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When amyloids become prions

Raimon Sabate1

  • 1Conformational Diseases Group; Department of Physical Chemistry; Faculty of Pharmacy; University of Barcelona (UB); Barcelona, Spain; Institut of Nanoscience and Nanotechnology of the University of Barcelona (IN2UB); Barcelona, Spain.

Prion
|May 17, 2014
PubMed

Insights

Protein aggregation causes conformational diseases like Alzheimer disease and prion diseases. Intrinsic cytotoxicity and aggregation nuclei per cell may explain differing infectious capacities of amyloid proteins.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Pathology

Background:

  • Conformational diseases involve protein aggregation into amyloid structures.
  • These diseases range from neurodegenerative disorders like Alzheimer disease (AD) to infectious transmissible spongiform encephalopathies (TSEs), collectively known as prion diseases.
  • The varying infectious potential of different amyloid-forming proteins remains unexplained.

Purpose of the Study:

  • To investigate the underlying factors determining the infectious capacity of amyloid proteins.
  • To explore the roles of intrinsic cytotoxicity and intracellular aggregation nucleation in prion disease transmission.

Main Methods:

  • This study proposes a theoretical framework based on existing literature and biochemical principles.
  • It analyzes the relationship between protein aggregation kinetics, cellular toxicity, and disease transmissibility.

Main Results:

  • Amyloid proteins share the potential to act as prions, facilitating cell-to-cell or organism-to-organism spread.
  • Intrinsic cytotoxicity and the number of aggregation nuclei within a cell are proposed as key determinants of an amyloid's infectious capacity.

Conclusions:

  • Understanding the factors governing amyloid infectivity is crucial for differentiating between various conformational diseases.
  • Intrinsic cytotoxicity and aggregation nucleation offer a potential explanation for the spectrum of prion disease infectivity.
Keywords:
Alzheimer diseaseCreutzfeldt–Jakob diseaseamyloidamyloid cytotoxicityamyloid transmissionpriontransmissible spongiform encephalopathy

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