Thoughts on mammalian prion strains

Folia Neuropathol. 2009;47(2):104-13.

Abstract

A plethora of prion strains can be propagated indefinitely in hosts homozygous for the PrP gene. Within the framework of the "protein-only" hypothesis, the strain-specific properties are enciphered in the conformation of the strain-associated PrPSc. Are these conformations codetermined by additional components, whose presence or absence within an infected cell could define the cell's competence to replicate a particular strain? Which cellular components, if any, contribute to the PrPC-to-PrPSc conversion in the cell? Many questions still remain to be answered in the field launched and nurtured by Carlton Gajdusek, to whom this essay is dedicated.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Humans
  • Prions / chemistry*
  • Prions / genetics*
  • Protein Conformation

Substances

  • Prions