Article
Autophagy and cell death of Purkinje cells overexpressing Doppel in Ngsk Prnp-deficient mice.
Brain pathology (Zurich, Switzerland) - 1 Jan 2010
Heitz Stéphane, Grant Nancy J, Leschiera Raphael, Haeberlé Anne-Marie, Demais Valérie, Bombarde Guy, Bailly Yannick
Abstract excerpt
In Ngsk prion protein (PrP)-deficient mice (NP(0/0)), ectopic expression of PrP-like protein Doppel (Dpl) in central neurons induces significant Purkinje cell (PC) death resulting in late-onset ataxia. NP(0/0) PC death is partly prevented by either knocking-out the apoptotic factor BAX or overexpressing the anti-apoptotic factor BCL-2 suggesting that apoptosis is involved in Dpl-induced death. In this study,...
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