Thoughts on mammalian prion strains

Charles Weissmann

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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2/2009
vol. 47

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abstract:

Thoughts on mammalian prion strains

Charles Weissmann

Folia Neuropathol 2009; 47 (2): 104-113

Online publish date: 2009/06/26

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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.

keywords:

CDI, conformation-dependent immunoassay, conformation templating, conformational population distribution, conversion, PrPC-to-PrPSc, CPA, cell panel assay, Gajdusek, glycoform, PMCA, protein misfolding cyclic amplification, protein-only, SSCA, standard scrapie cell assay, strains, TSE, transmissible spongiform encephalopathy