Molecular pathogenesis of sporadic prion diseases in man

Figures & data

Table 1. Etiologic classification of human prion diseases

Etiology	Mechanism	Disease
Sporadic	Somatic mutation or spontaneous conversion of PrP^C into PrP^Sc ?	Sporadic Creutzfeldt-Jakob disease
	Somatic mutation or spontaneous conversion of PrP^C into PrP^Sc ?	Sporadic fatal insomnia
	Unknown	Variable protease-sensitive prionopathy
Hereditary	Germ-line mutations in the PRNP gene	Familial Creutzfeldt-Jakob disease
	Germ-line mutations in the PRNP gene	Fatal familial insomnia
	Germ-line mutations in the PRNP gene	Gerstmann–Sträussler-Scheinker disease
Acquired	Infection through ritualistic cannibalism	Kuru
	Zoonotic infection with bovine prions	Variant Creutzfeldt-Jakob disease
	Infection from prion-contaminated human growth hormone, dura mater grafts, etc.	Iatrogenic Creutzfeldt-Jakob disease

Figure 1. The relationship between duration of the disease, (left panel) concentration, and (right panel) conformational stability of sPrP^Sc conformers in sCJD patients with different WB patterns and PRNP gene polymorphisms.⁹² The stability of PrPSc was determined with CDI before and after protease treatment and the change is expressed as ΔGdn HCl_1/2.⁹² The symbols are mean ± SEM in each sCJD group.

Kim C, Haldiman T, Cohen Y, Chen W, Blevins J, Sy MS, et al. Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate. PLoS Pathog 2011; 7:e1002242; http://dx.doi.org/10.1371/journal.ppat.1002242; PMID: 21931554

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