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Fatal insomnia
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==Research== Still with unclear benefit in humans, a number of treatments have had tentative success in slowing disease progression in animal models, including [[pentosan polysulfate]], [[mepacrine]], and [[amphotericin B]].<ref name="GARD2019" /> {{As of|2016}}, a study investigating [[doxycycline]] is being carried out.<ref name="GARD2019" /><ref>{{cite journal | vauthors = Forloni G, Tettamanti M, Lucca U, Albanese Y, Quaglio E, Chiesa R, Erbetta A, Villani F, Redaelli V, Tagliavini F, Artuso V, Roiter I | title = Preventive study in subjects at risk of fatal familial insomnia: Innovative approach to rare diseases | journal = Prion | volume = 9 | issue = 2 | pages = 75β79 | date = 21 May 2015 | pmid = 25996399 | pmc = 4601344 | doi = 10.1080/19336896.2015.1027857 }}</ref> In 2009, a mouse model was made for FFI. These mice expressed a humanized version of the PrP protein that also contains the'' D178N'' FFI mutation.<ref>{{cite journal | vauthors = Jackson WS, Borkowski AW, Faas H, Steele AD, King OD, Watson N, Jasanoff A, Lindquist S | title = Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice | journal = Neuron | volume = 63 | issue = 4 | pages = 438β450 | date = August 2009 | pmid = 19709627 | pmc = 2775465 | doi = 10.1016/j.neuron.2009.07.026 }}</ref> These mice appear to have progressively fewer and shorter periods of uninterrupted sleep, damage in the [[thalamus]], and early deaths, similar to humans with FFI.{{citation needed|date=June 2022}} The Prion Alliance was established by husband and wife duo Eric Minikel and Sonia Vallabh after Vallabh's mother was diagnosed with the fatal disease.<ref>{{cite magazine | vauthors = Clancy K |title=One Couple's Tireless Crusade to Stop a Genetic Killer |url=https://www.wired.com/story/sleep-no-more-crusade-genetic-killer/ |magazine=Wired |date=15 January 2019 }}</ref> They conduct research at the [[Broad Institute]] to develop therapeutics for human prion diseases. Other research interests involve identifying [[biomarker]]s to track the progression of prion disease in living people.<ref>{{Cite web|url=https://www.broadinstitute.org/bios/sonia-vallabh|title=Sonia Vallabh|date=2015-08-20|website=Broad Institute|language=en|access-date=2019-01-21}}{{self-published inline|date=June 2024}}</ref><ref>{{Cite web|url=http://www.prionalliance.org/|title=Prion Alliance|website=www.prionalliance.org|access-date=2019-01-21}}{{self-published inline|date=June 2024}}</ref>
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