NR ALLD
AU Tateishi,J.; Kitamoto,T.; Hoque,M.Z.; Furukawa,H.
TI Experimental transmission of Creutzfeldt-Jakob disease and related diseases to rodents
QU Neurology 1996 Feb; 46(2): 532-7
PT journal article
AB Sporadic Creutzfeldt-Jakob disease (CJD) with 129M/M, and iatrogenic and familial CJD with E200K and M232R, showed similar clinicopathologic features, a synaptic type deposition of PrPcJD, and high transmission frequencies to mice. Sporadic patients with 129M/V or 129V/V, and mutation cases with V180I, showed slightly different features and low or null transmission frequencies to mice. Hereditary cases with P102L, P105L, A117V, Y145stop, and insertions had different features but all demonstrated a long clinical duration and the presence of PrP plaques. The experimental transmission to mice of these mutant forms was difficult, except for one-third of the cases with P102L. CJD and related diseases, even those that are hereditary, may thus be divided into two different groups, those that are easily transmissible and those that are either difficult to transmit or nontransmissible.
IN Die Autoren übertrugen infektiöses Material von erblichen, iatrogenen und sogenannten sporadischen Creutzfeldt-Jakob-Patienten mit unterschiedlichen Mutationen oder Allelpaarvarianten auf Mäuse und stellten in Abhängigkeit von der genetischen Ausstattung der Patienten sehr verschiedene Übertragungseffizienzen bzw. Inkubationszeiten fest.
ZR 31
MH Adult; Age of Onset; Aged; Animal; Brain Tissue Transplantation/adverse effects; Case Report; Codon; Comparative Study; Creutzfeldt-Jakob Syndrome/*genetics/physiopathology/*transmission; Electroencephalography; Female; Genotype; Guinea Pigs; Hamsters; Homozygote; Human; Iatrogenic Disease; Male; Mice; Middle Age; Point Mutation; Prions/analysis; Rats; Support, Non-U.S. Gov't; Time Factors; *Zoonoses
AD Department of Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
SP englisch
PO USA