[HTML][HTML] Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B …
B Drisaldi, J Coomaraswamy, P Mastrangelo… - Journal of Biological …, 2004 - ASBMB
The PrP-like Doppel (Dpl) protein causes apoptotic death of cerebellar neurons in
transgenic mice, a process prevented by expression of the wild type (wt) cellular prion
protein, PrP C. Internally deleted forms of PrP C resembling Dpl such as PrPΔ32–121
produce a similar PrP C-sensitive pro-apoptotic phenotype in transgenic mice. Here we
demonstrate that these phenotypic attributes of wt Dpl, wt PrP C, and PrPΔ132–121 can be
accurately recapitulated by transfected mouse cerebellar granule cell cultures. This system …
transgenic mice, a process prevented by expression of the wild type (wt) cellular prion
protein, PrP C. Internally deleted forms of PrP C resembling Dpl such as PrPΔ32–121
produce a similar PrP C-sensitive pro-apoptotic phenotype in transgenic mice. Here we
demonstrate that these phenotypic attributes of wt Dpl, wt PrP C, and PrPΔ132–121 can be
accurately recapitulated by transfected mouse cerebellar granule cell cultures. This system …