Article
Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 11 May 2005
Radovanovic Ivan, Braun Nathalie, Giger Olivier T, Mertz Kirsten, Miele Gino, Prinz Marco, Navarro Beatriz, Aguzzi Adriano
Abstract excerpt
The cellular prion protein PrP(C) confers susceptibility to transmissible spongiform encephalopathies, yet its normal function is unknown. Although PrP(C)-deficient mice develop and live normally, expression of amino proximally truncated PrP(C) (DeltaPrP) or of its structural homolog Doppel (Dpl) causes cerebellar degeneration that is prevented by coexpression of full-length PrP(C). We now report that mice...
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