Review article<br>

Prion protein (PrP) deposits in the tectum of experimental Gerstmann-Sträussler-Scheinker disease following intraocular inoculation

Pawel P. Liberski; Johannes A. Hainfellner; Beata Sikorska; Herbert Budka

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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1/2012
vol. 50

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Review article
Prion protein (PrP) deposits in the tectum of experimental Gerstmann-Sträussler-Scheinker disease following intraocular inoculation

Pawel P. Liberski

,

Johannes A. Hainfellner

,

Beata Sikorska

,

Herbert Budka

Folia Neuropathol 2012; 50 (1): 85-88

Online publish date: 2012/03/30

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The abnormal misfolded isoform of prion protein (PrP^d; “d” for disease) is considered as a surrogate marker for infectivity in the transmissible spongiform encephalopathies (TSEs) or prion diseases, including Creutzfeldt-Jakob disease (CJD). In this experiment, we used intraocular inoculation to study PrP^d deposition in the visual system of the brain of mice infected with the Fujisaki (K.Fu) strain of Gerstmann-Sträussler-Scheinker (GSS) disease. We report here that PrP^d is deposited in the superior colliculus following contralateral intraocular inoculation and thus follows neuronal connections when it spreads into the brain. Until 26 weeks postinoculation, no PrP^d-specific immunostaining was observed in the brain. At 27 weeks postinoculation, PrP^d targeted to the contralateral superior colliculus as delicate granular synaptic deposits located in the superficial part of this structure. As already reported, a few spongiform vacuoles were visible in the same area by conventional H&E staining. In several other sections, vacuoles were visible but no PrPd staining could be detected.

keywords:

Review article Prion protein (PrP) deposits in the tectum of experimental Gerstmann-Sträussler-Scheinker disease following intraocular inoculation

Pawel P. Liberski , Johannes A. Hainfellner , Beata Sikorska , Herbert Budka

prion, prion diseases, Gerstmann-Sträussler-Scheinker disease, spreading of prions

Review article
Prion protein (PrP) deposits in the tectum of experimental Gerstmann-Sträussler-Scheinker disease following intraocular inoculation

Pawel P. Liberski

,

Johannes A. Hainfellner

,

Beata Sikorska

,

Herbert Budka