Prions in the environment

Saunders, Samuel E.; Bartelt–Hunt, Shannon L.; Bartz, Jason C.

doi:10.4161/pri.2.4.7951

Cited by 80 publications

(37 citation statements)

References 103 publications

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“…The diseases can be transmitted within and between species by several mechanisms, including ingestion, iatrogenic transmission and blood transfusion [2]. The possibility for spreading through environmental exposure cannot be discounted, because the infectious agent (called a prion) persists in the environment for many years [3]. …”

Section: Prions and Prion Diseasesmentioning

confidence: 99%

The intricate mechanisms of neurodegeneration in prion diseases

Soto¹,

Satani²

2011

Trends in Molecular Medicine

132

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Prion diseases are a group of infectious neurodegenerative diseases with an entirely novel mechanism of transmission, involving a protein-only infectious agent that propagates the disease by transmitting protein conformational changes. The disease results from extensive and progressive brain degeneration. The molecular mechanisms involved in neurodegeneration are not entirely known but involve multiple processes operating simultaneously and synergistically in the brain, including spongiform degeneration, synaptic alterations, brain inflammation, neuronal death and the accumulation of protein aggregates. Here, we review the pathways implicated in prion-induced brain damage and put the pieces together into a possible model of neurodegeneration in prion disorders. A more comprehensive understanding of the molecular basis of brain degeneration is essential to develop a much needed therapy for these devastating diseases.

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Section: Prions and Prion Diseasesmentioning

confidence: 99%

The intricate mechanisms of neurodegeneration in prion diseases

Soto¹,

Satani²

2011

Trends in Molecular Medicine

132

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“…Studies proved that CWD can be horizontally transmitted among cervids either by direct contact with infected animals [43], their secreta [32], [33], [38], [44] or indirectly through the environment they inhabit [39], [45]. As observed in other animal TSEs, even cervids in the subclinical phase of disease harbor transmissible infectious prions [32], [39], [46]–[49].…”

Section: Introductionmentioning

confidence: 99%

Mother to Offspring Transmission of Chronic Wasting Disease in Reeves’ Muntjac Deer

et al. 2013

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The horizontal transmission of prion diseases has been well characterized in bovine spongiform encephalopathy (BSE), chronic wasting disease (CWD) of deer and elk and scrapie of sheep, and has been regarded as the primary mode of transmission. Few studies have monitored the possibility of vertical transmission occurring within an infected mother during pregnancy. To study the potential for and pathway of vertical transmission of CWD in the native cervid species, we used a small cervid model–the polyestrous breeding, indoor maintainable, Reeves’ muntjac deer–and determined that the susceptibility and pathogenesis of CWD in these deer reproduce that in native mule and white-tailed deer. Moreover, we demonstrate here that CWD prions are transmitted from doe to fawn. Maternal CWD infection also appears to result in lower percentage of live birth offspring. In addition, evolving evidence from protein misfolding cyclic amplification (PMCA) assays on fetal tissues suggest that covert prion infection occurs in utero. Overall, our findings demonstrate that transmission of prions from mother to offspring can occur, and may be underestimated for all prion diseases.

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“…The use introduction of proteins into a soil matrix can result in their degradation via several mechanisms including microbial digestion or abiotic degradation. 27,28 Autoclaving is known to kill microbes and reduce protease activity. 29 The data presented here indicate that the temperature-dependent cleavage of the N-terminal region of PrP TSE that is observed within the first 11 d of incubation is due to abiotic rather than microbial degradation.…”

Section: Methodsmentioning

confidence: 99%