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Loss of Cerebellar Granule Neurons Is Associated With Punctate but Not With Large Focal Deposits of Prion Protein in Creutzfeldt-Jakob Disease
2009
Journal of Neuropathology and Experimental Neurology
Whether aggregates of prion protein (PrP) reflect neurotoxicity or are neuroprotective in prion diseases is unclear. To address this question, we performed a clinicopathologic study of cerebellar granular neurons in 100 patients affected with sporadic Creutzfeldt-Jakob disease (CJD). There was significant loss of these neurons in the subset of cases with Val/Val genotype at PRNP Codon 129 and Molecular Isotype 2 of abnormal PrP (sporadic CJD-VV2) (n = 32) compared with both the other CJD
doi:10.1097/nen.0b013e3181af7f23
pmid:19606064
fatcat:5kduhw2fgrbj5gfemxm6o5cje4