Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice.
Kuru provides our principal experience of an epidemic human prion disease and primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. Kuru was transmitted by the practice of consuming dead relatives as a mark of respect and mourning (transumption). To date, detailed information of the prion strain type propagated in kuru has been lacking. Here, we directly compare the transmission properties of kuru prions with sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease (CJD) prions in Prnp-null transgenic mice expressing human prion protein and in wild-type mice. Molecular and neuropathological data from these transmissions show that kuru prions are distinct from variant CJD and have transmission properties equivalent to those of classical (sporadic) CJD prions. These findings are consistent with the hypothesis that kuru originated from chance consumption of an individual with sporadic CJD.
Wadsworth
Jonathan D F
JD
Medical Research Council Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Joiner
Susan
S
Linehan
Jacqueline M
JM
Desbruslais
Melanie
M
Fox
Katie
K
Cooper
Sharon
S
Cronier
Sabrina
S
Asante
Emmanuel A
EA
Mead
Simon
S
Brandner
Sebastian
S
Hill
Andrew F
AF
Collinge
John
J
D000818:Animals; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology / Q000635:transmission*; D006801:Humans; D015151:Immunoblotting; D007729:Kuru / Q000473:pathology / Q000635:transmission*; D051379:Mice; D008822:Mice, Transgenic; D010641:Phenotype; D018620:PrPSc Proteins / Q000378:metabolism; D011328:Prions / Q000378:metabolism*
D018620:PrPSc Proteins; D011328:Prions