title: Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice.
abstract: 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.
authors.affiliation: 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.
authors.forename: Jonathan D F
authors.initials: JD
authors.lastname: Wadsworth
authors.forename: Susan
authors.initials: S
authors.lastname: Joiner
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Linehan
authors.forename: Melanie
authors.initials: M
authors.lastname: Desbruslais
authors.forename: Katie
authors.initials: K
authors.lastname: Fox
authors.forename: Sharon
authors.initials: S
authors.lastname: Cooper
authors.forename: Sabrina
authors.initials: S
authors.lastname: Cronier
authors.forename: Emmanuel A
authors.initials: EA
authors.lastname: Asante
authors.forename: Simon
authors.initials: S
authors.lastname: Mead
authors.forename: Sebastian
authors.initials: S
authors.lastname: Brandner
authors.forename: Andrew F
authors.initials: AF
authors.lastname: Hill
authors.forename: John
authors.initials: J
authors.lastname: Collinge
mesh_terms: 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*
chemical_list: D018620:PrPSc Proteins; D011328:Prions