Mice Study by Medical Research Council Prion Unit at UCL:
In studying the harmful effects of Kuru, researchers at the UCL Institute of Neurology in the U.K. injected transgenic mice that were bred for increased receptivity to the Kuru disease [1] with two variants of the prion protein gene (PRNP). These included the G127V PRNP, a gene that was positively selected for during the Kuru outbreak, and the more commonly known V127 on the M129V PRNP allele, to test how each would affect resistance to Kuru. The researchers found that mice injected with both variants received full resistance to the heterozygous form of Kuru and Classical Creutzfeldt-Jakob disease (CJD), but were still susceptible to infection by non-classical, or variant CJD. This strain of CJD was not found in the Fore people, however it was found in humans from other regions in contact with bovine spongiform encephalopathy prions. Mice injected with V127 alone, however, were resistant to all variations of the prion protein gene, demonstrating that just one difference in amino acid on the G127V PrnP, from Glycine to Valine, was capable of granting resistance to the disease [2]
Pop Culture/Media References:
Book Of Eli:
The post apocalyptic setting featured in The Book of Eli details a world where basic human necessities, such as food and water, are marginally available, as well as rationed by Carnegie, the prominent antagonist throughout the entirety of the film. Eli, a god-fearing man who finds the last copy of the Bible and vows to spread it’s holy message throughout the western world, stumbles across an old married couple clinging to survive in these harsh times, who also suffer from “shaking hands”. He is deeply disturbed when he comes to notice the graveyard located behind their home, ultimately realizing that their tremors are a result of their resort to feeding off of human remains infected by Kuru.[3]
Scream Queens:
In the second season of the television series, Scream Queens, Dean Cathy Munsch (Jamie Lee Curtis) purchases the C.U.R.E. Institute Hospital, insisting her motives were solely based on her drive to try something spontaneous. Zayday, one of the medical students at the hospital suspects otherwise, and researches Munsch’s symptoms, such as her trembling, headaches, and difficulty walking. Munsch finally confides in Zayday, explaining how she believes she has contracted Kuru during her visit to New Guinea, where she attended a funeral and was unknowingly served human organs infected with the disease.[4]
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- ^ "Study reveals gene mutation that confers resistance against prion disease". Retrieved 2016-11-15.
- ^ Asante, Emmanuel A.; Smidak, Michelle; Grimshaw, Andrew; Houghton, Richard; Tomlinson, Andrew; Jeelani, Asif; Jakubcova, Tatiana; Hamdan, Shyma; Richard-Londt, Angela (2015-06-25). "A naturally occurring variant of the human prion protein completely prevents prion disease". Nature. 522 (7557): 478–481. doi:10.1038/nature14510. ISSN 0028-0836. PMC 4486072. PMID 26061765.
- ^ "The Book of Eli: Movie Review". CBN.com (beta). Retrieved 2016-11-15.
- ^ Walsh, Megan. "What Disease Does Dean Munsch Have On 'Scream Queens'? It's Incredibly Rare". Romper. Retrieved 2016-11-15.