Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions

Article Abstract:

The role of prion protein (PrP) in scrapie pathogenesis and prion propagation was investigated. Transgenic mice that express truncated PrP were developed and characterized to examine the structure-activity relationship of PrP. Results show that Prp with no residues 32-121 or 32-134 but with shorter deletions cause severe ataxia and neuronal death specific to the granular layer of the cerebellum as early as one to three months after birth. It was speculated that the truncated PrP may compete with some other molecule with a PrP-like function for a common ligand.

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Soluble dimeric prion protein binds PrP(sup)Sc in vivo and antagonizes prion disease

Article Abstract:

Research demonstrates that in wild-type mice soluble cellular dimeric prion protein, prepared by fusion to an immunoglobulin, stalls the pathogenic cellular prion protein, PrP(sup)Sc, thereby preventing the onset of scrapie disease following inoculation with infective prions.

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Mammalian prion biology: one century of evolving concepts

Article Abstract:

The devastation caused by prions is surveyed. The representation of prions as a fundamental mechanisms for cross-talk between proteins is discussed.

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