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Prion Disease

Distinct PrP patterns in cerebellum may indicate familial CJD

Published in Pain and Central Nervous System Week, August 4th, 2003

Distinct patterns of prion protein deposits in the cerebellum may indicate familial Creutzfeldt-Jakob disease.

According to published research from Austria, "We have compared the immunomorphological spectrum of the deposition of the disease-associated prion protein (PrPSc) in the cerebral and cerebellar cortex of 32 Creutzfeldt-Jakob disease (013) patients with the PrP gene (PRNP) E200K mutation to 45 sporadic CJD and 14 other genetic prion disease cases. PrP deposits correlate with the genotype at the methionine/valine (MV) polymorphic codon 129.

"While the diffuse/synaptic and patchy/perivacuolar PrP deposits and PrP plaques have a similar...

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