Infectious prion diseases in humans: Cannibalism, iatrogenicity and zoonoses

Developing Therapeutics for PrP Prion Diseases

“…In addition, a number of well-documented CJD cases have an iatrogenic origin, the vast majority of these stemming from the use of cadaver-derived tissues that, without clinicians’ knowledge, contained CJD prions. Additionally, a handful of cases have also occurred from the reuse of incompletely decontaminated surgical instruments (Haïk et al 2014). …”

Brain and immune system: KURU disease a toxicological process?

“…Compared to its normal counterpart, PrP(Sc) is β-sheet enriched and aggregated and its propagation is based on an autocatalytic conversion process. Here, we review and discuss how genetic factors interplay with strain properties and route of transmission to in luence disease susceptibility, incubation period and phenotypic expression in the light of the kuru epidemics due to ritual endocannibalism, the various series iatrogenic diseases secondary to extractive growth hormone treatment or dura mater graft and the epidemics of variant Creutzfeldt-Jakob disease linked to dietary exposure to the agent of bovine spongiform encephalopathy [3].…”

“…The peripheral pathogenesis of these diseases have been extensively studied using animal models, as mice are naturally susceptible to both sheep scrapie and bovine spongiform encephalopathy (BSE). Following natural peripheral exposure to prion agents, infection is usually sequestered to lymphoid organs prior to invasion of the nervous system (termed neuroinvasion) (2,3). In the absence of local draining lymphoid tissue, subsequent circulation to other lymphoid organs and neuroinvasion are ultimately blocked (4,5).…”

“…This applies to α-synuclein in models of PD, DLB and MSA, as well as Tau in models of Alzheimer's disease; mutant huntingtin in Huntington's disease models, and mutant SOD in amyotrophic lateral sclerosis (ALS) models [2,3]. The behavior of these diseaserelated proteins has been called "prion-like".…”

La maladie de Parkinson est-elle une maladie à prion ?

“…Ces maladies peuvent se transmettre, directement ou indirectement, d'une personne à l'autre ») (http:// www.who.int/topics/infectious_diseases/fr/il), il faut souligner que, chez l'homme et contrairement à certaines maladies animales comme la maladie du dé pé rissement chronique des cervidé s, elles ne sont pas contagieuses. Les rares cas de transmission interhumaine, depuis la disparition du kuru, ré sultent de contaminations accidentelles en rè gle iatrogè ne [4]. Un prion est donc un agent transmissible accidentellement ou expé rimentalement dont l'infectivité peut ê tre titré e. Il faut bien les distinguer de ce qui a é té appelé par facilité de langage « prions de levure » ou « prions physiologiques » en ré fé rence au mé canisme molé culaire gouvernant la ré plication de ces agents et aux proprié té s de la proté ine prion.…”