Autophagy and cell death of Purkinje cells overexpressing Doppel in Ngsk Prnp-deficient mice.
Strasbourg, France. In Brain Pathol, 2010
In this study, Western blotting and immunohistofluorescence show that both before and during significant PC loss, the scrapie-responsive gene 1 (Scrg1)--potentially associated with autophagy--and the autophagic markers LC3B and p62 increased in the NP(0/0) PCs whereas RT-PCR shows stable mRNA expression, suggesting that the degradation of autophagic products is impaired in NP(0/0) PCs.
Doppel induces autophagic stress in prion protein-deficient Purkinje cells.
Strasbourg, France. In Autophagy, 2009
These neurons display increased amounts of several autophagy-related molecules such as the scrapie-responsive gene one (Scrg1), LC3B-II and p62 without showing any changes in mRNA expression; in addition, autolysosomes accumulate in all neuronal compartments including axon terminals.
Characterization of the human analogue of a Scrapie-responsive gene.
Villejuif, France. In J Biol Chem, 1998
We have recently described a novel mRNA denominated ScRG-1, the level of which is increased in the brains of Scrapie-infected mice (Dandoy-Dron, F., Guillo, F., Benboudjema, L., Deslys, J.-P., Lasmézas, C., Dormont, D., Tovey, M. G., and Dron, M. (1998) J. Biol.