Comparative neuropathology of Kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host.
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.
McLean
C A
CA
Department of Pathology, The University of Melbourne, Victoria, Australia. c.mclean@pathology.unimelb.edu.au
Ironside
J W
JW
Alpers
M P
MP
Brown
P W
PW
Cervenakova
L
L
Anderson
R M
RM
Masters
C L
CL
D000293:Adolescent; D000328:Adult; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000473:pathology*; D004247:DNA / Q000032:analysis; D005260:Female; D005838:Genotype; D006801:Humans; D007124:Immunoenzyme Techniques; D007729:Kuru / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011328:Prions / Q000235:genetics*
D011328:Prions; D004247:DNA