NR AHQQ
AU MacDonald,S.T.; Sutherland,K.; Ironside,J.W.
TI Prion protein genotype and pathological phenotype studies in sporadic Creutzfeldt-Jakob disease
QU Neuropathology and Applied Neurobiology 1996 Aug; 22(4): 285-92
PT journal article
AB A comparative semi-automated morphometric study was performed on the distribution of prion protein, spongiform change and astrocytosis in the brains of nine cases of sporadic Creutzfeldt-Jakob disease of differing genotype at the methionine-valine polymorphism at codon 129 of the prion protein gene. Custom-designed image analysis software was used to produce objective figures for each of the different pathological features throughout 13 different areas of the brain used for analysis. A significant positive correlation was observed between prion protein deposition and astrocytosis in all cases and no significant correlation was observed between spongiform change and prion protein deposition. Different patterns of pathology were found to relate to codon 129 genotype; valine homozygosity favoured the targeting of pathology to deep grey matter structures, while methionine homozygosity favoured cortical targeting of pathology. These results provide evidence that prion protein deposition is closely associated with an astrocytic reaction and suggest that codon 129 genotype may influence the pathological phenotype.
IN Bei 9 britischen Fällen "sporadischer" Creutzfeldt-Jakob-Krankheit korrelierte die Menge der Prionprotein-Ablagerungen mit der Astrozytose, jedoch nicht mit der Spongiose. Die pathologischen Schädigungsmuster an 13 untersuchten Hirnregionen unterschieden sich signifikant in Abhängigkeit davon, ob die Patienten am Codon 129 Met/Met, Met/Val oder Val/Val besaßen. Während Valin-homozygote Patienten besonders in den tieferliegenden grauen Schichten geschädigt waren, überwogen bei den Methionin-homozygoten Patienten Schäden in der Hirnrinde.
ZR 25
MH Aged; Aged, 80 and over; Brain/pathology; Brain Chemistry/physiology; Cerebral Cortex/metabolism/pathology; Codon; Creutzfeldt-Jakob Syndrome/*genetics/metabolism/*pathology; Female; Human; Image Processing, Computer-Assisted; Immunohistochemistry; Male; Middle Age; Phenotype; Prions/*genetics/metabolism; Sequence Analysis, DNA; Support, Non-U.S. Gov't; Tissue Fixation
AD National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, UK
SP englisch
PO England