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Molecular analysis of prion protein (PrP) and glial fibrillary acidic protein (GFAP) transcripts in experimental Creutzfeldt Jakob disease in mice

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Kordek R., Liberski P. P., Yanagihra R., Isaacson S., Gajdusek D. C.
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Prion protein (PrPsc) which accumulates in the brains affected with subacute spongiform encephalopathies (SSE) is altered isoform of normal, cellular isoform (PrPc), and PrP deposition is accompanied with spongiosis and astrogliosis. To find the amounts of PrP and GFAP transcripts during progression of experimental Creutzfeldt Jakob disease we performed comparative RT PCR on the terminally sick mice brains, 22 weeks following inoculation with Fujisaki strain of CJD agent, and on control brains. The intensity of bands for PrP mRNA and control beta actin were similar for infected and uninfected brains, while amounts of transcripts for GFAP increased as for cytokines released by glial cells TNF-alpha and IL-1 alpha. This study supports thesis that PrPc to PrPsc conversion is post translational process not related to PrP overproduction. Increased amounts of GFAP mRNA during the course of the disease correlated with astrocytosis estimated by immunohistochemistry with anti GFAP antibody.
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