Comparative Neuropathology of Kuru with the New Variant of Creutzfeldt‐Jakob Disease: Evidence for Strain of Agent Predominating Over Genotype of Host
- 1 July 1998
- journal article
- Published by Wiley in Brain Pathology
- Vol. 8 (3) , 429-437
- https://doi.org/10.1111/j.1750-3639.1998.tb00165.x
Abstract
The three major influences on the phenotype of the transmissible spongiform encephalopathies are believed to be strain of agent, route of infection and host genotype. We have compared the pathologic profiles and genotypes of the new variant of Creutzfeldt-Jakob disease (vCJD) and kuru. The comparison reveals that there are distinct lesional differences particularly in the prion protein (PrP) load and distribution as seen by immunohistochemistry. The clinico-pathologic phenotypes and the genotypes of these two diseases are sufficiently different to suggest that the strain of agent may play a greater role than any presumptive common route of peripherally acquired infection.Keywords
This publication has 37 references indexed in Scilit:
- Typing prion isoformsNature, 1997
- Prion protein genotype and pathological phenotype studies in sporadic Creutzfeldt‐Jakob diseaseNeuropathology and Applied Neurobiology, 1996
- Molecular basis of phenotypic variability in sporadc creudeldt‐jakob diseaseAnnals of Neurology, 1996
- A new variant of Creutzfeldt-Jakob disease in the UKPublished by Elsevier ,1996
- The Original Gerstmann‐Sträussler‐Scheinker Family of Austria: Divergent Clinicopathological Phenotypes but Constant PrP GenotypeBrain Pathology, 1995
- The Nosology of Creutzfeldt‐Jakob Disease and Conditions Related to the Accumulation of PrPCJD in the Nervous SystemBrain Pathology, 1995
- Neuropathological phenotype and ‘prion protein’ genotype correlation in sporadic Creutzfeldt-Jakob diseaseNeuroscience Letters, 1994
- Prion protein immunocytochemistry: reliable protocols for the investigation of Creutzfeldt–Jakob diseaseNeuropathology and Applied Neurobiology, 1994