Prion strain diversity

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Prion diseases affect a wide range of mammal species and are caused by a misfolded selfpropagating isoform (PrPSc) of the normal prion protein (PrPC). Distinct strains of prions exist and are operationally defined by differences in a heritable phenotype under controlled experimental transmission conditions. Prion strains can differ in incubation period, clinical signs of disease, tissue tropism, and host range. The mechanism by which a protein-only pathogen can encode strain diversity is only beginning to be understood. The prevailing hypothesis is that prion strain diversity is encoded by strain-specific conformations of PrPSc; however, strain-specific cellular cofactors have been identified in vitro that may also contribute to prion strain diversity. Although much progress has been made on understanding the etiological agent of prion disease, the relationship between the strain-specific properties of PrPSc and the resulting phenotype of disease in animals is poorly understood.

Original languageEnglish (US)
Article numbera024349
JournalCold Spring Harbor perspectives in medicine
Volume6
Issue number12
DOIs
StatePublished - 2016

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Prions
Prion Diseases
Phenotype
Animal Diseases
Tropism
Host Specificity
PrPC Proteins
Mammals
Protein Isoforms
Pathogens
Conformations
Animals
Proteins
Tissue

All Science Journal Classification (ASJC) codes

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Prion strain diversity. / Bartz, Jason C.

In: Cold Spring Harbor perspectives in medicine, Vol. 6, No. 12, a024349, 2016.

Research output: Contribution to journalArticle

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