Experimental models of human prion diseases and prion strains

Abigail B. Diack, Jason C. Bartz

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Prion strains occur in natural prion diseases, including prion diseases of humans. Prion strains can correspond with differences in the clinical signs and symptoms of disease and the distribution of prion infectivity in the host and are hypothesized to be encoded by strain-specific differences in the conformation of the disease-specific isoform of the host-encoded prion protein, PrPTSE. Prion strains can differ in biochemical properties of PrPTSE that can include the relative sensitivity to digestion with proteinase K and conformational stability in denaturants. These strain-specific biochemical properties of field isolates are maintained upon transmission to experimental animal models of prion disease. Experimental human models of prion disease include traditional and gene-targeted mice that express endogenous PrPC. Transgenic mice that express different polymorphs of human PrPC or mutations in human PrPC that correspond with familial forms of human prion disease have been generated that can recapitulate the clinical, pathologic, and biochemical features of disease. These models aid in understanding disease pathogenesis, evaluating zoonotic potential of animal prion diseases, and assessing human-to-human transmission of disease. Models of sporadic or familial forms of disease offer an opportunity to define mechanisms of disease, identify key neurodegenerative pathways, and assess therapeutic interventions.

Original languageEnglish (US)
Title of host publicationHandbook of Clinical Neurology
PublisherElsevier B.V.
Pages69-84
Number of pages16
DOIs
StatePublished - Jan 1 2018

Publication series

NameHandbook of Clinical Neurology
Volume153
ISSN (Print)0072-9752
ISSN (Electronic)2212-4152

Fingerprint

Prion Diseases
Prions
Theoretical Models
Endopeptidase K
Animal Diseases
Zoonoses
Transgenic Mice
Signs and Symptoms
Digestion
Protein Isoforms
Animal Models
Mutation
Genes

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Diack, A. B., & Bartz, J. C. (2018). Experimental models of human prion diseases and prion strains. In Handbook of Clinical Neurology (pp. 69-84). (Handbook of Clinical Neurology; Vol. 153). Elsevier B.V.. https://doi.org/10.1016/B978-0-444-63945-5.00004-0

Experimental models of human prion diseases and prion strains. / Diack, Abigail B.; Bartz, Jason C.

Handbook of Clinical Neurology. Elsevier B.V., 2018. p. 69-84 (Handbook of Clinical Neurology; Vol. 153).

Research output: Chapter in Book/Report/Conference proceedingChapter

Diack, AB & Bartz, JC 2018, Experimental models of human prion diseases and prion strains. in Handbook of Clinical Neurology. Handbook of Clinical Neurology, vol. 153, Elsevier B.V., pp. 69-84. https://doi.org/10.1016/B978-0-444-63945-5.00004-0
Diack AB, Bartz JC. Experimental models of human prion diseases and prion strains. In Handbook of Clinical Neurology. Elsevier B.V. 2018. p. 69-84. (Handbook of Clinical Neurology). https://doi.org/10.1016/B978-0-444-63945-5.00004-0
Diack, Abigail B. ; Bartz, Jason C. / Experimental models of human prion diseases and prion strains. Handbook of Clinical Neurology. Elsevier B.V., 2018. pp. 69-84 (Handbook of Clinical Neurology).
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