Transmission of Prions from Mule Deer and Elk with Chronic Wasting Disease to Transgenic Mice Expressing Cervid PrP

Browning, Shawn; Mason, Gary L.; Seward, Tanya; Green, Mike; Eliason, Gwyneth A. J.; Mathiason, Candace K.; Miller, Michael W.; Williams, Elizabeth; Hoover, Ed; Telling, Glenn C.

doi:10.1128/jvi.78.23.13345-13350.2004

Cited by 180 publications

(249 citation statements)

References 44 publications

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“…Two other 'absolute' species barriers in mice have recently been broken. Expression of mink PrP [206] and cervid PrP [30,82,113,117,134,188] in transgenic mice render them susceptible to TME and CWD, respectively. It is noteworthy that the use of transgenic mice overexpressing mouse PrP C also allowed the propagation of the CWD agent [179].…”

Section: 'Absolute' Species Barriermentioning

confidence: 99%

“…This apparent lack of species barrier after homotypic transmission -i.e. when the host expresses a PrP gene identical to that of the infecting species -has led to the development of a long list of mice transgenic for sheep [60,197,203], bovine [20,41,48,175], human [5,22,113,190], cervid [30,82,113,117,134,188] and mink [206] PrP. Most of such lines were obtained by additional transgenesis, and have an endogenous PrP null (PrP 0/0 ) background, in order to avoid any interfering effect of the resident murine PrP gene [39,190].…”

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confidence: 99%

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Prion agent diversity and species barrier

2008

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-Mammalian prions are the infectious agents responsible for transmissible spongiform encephalopathies (TSE), a group of fatal, neurodegenerative diseases, affecting both domestic animals and humans. The most widely accepted view to date is that these agents lack a nucleic acid genome and consist primarily of PrP Sc , a misfolded, aggregated form of the host-encoded cellular prion protein (PrP C ) that propagates by autocatalytic conversion and accumulates mainly in the brain. The BSE epizooty, allied with the emergence of its human counterpart, variant CJD, has focused much attention on two characteristics that prions share with conventional infectious agents. First, the existence of multiple prion strains that impose, after inoculation in the same host, specific and stable phenotypic traits such as incubation period, molecular pattern of PrP Sc and neuropathology. Prion strains are thought to be enciphered within distinct PrP Sc conformers. Second, a transmission barrier exists that restricts the propagation of prions between different species. Here we discuss the possible situations resulting from the confrontation between species barrier and prion strain diversity, the molecular mechanisms involved and the potential of interspecies transmission of animal prions, including recently discovered forms of TSE in ruminants.prion / strain / misfolding / species barrier / PrP Table of Mammalian prion diseases or transmissible spongiform encephalopathies (TSE) form a group of related, invariably fatal neurodegenerative disorders of both animals and humans. The brain pathology consists of spongiosis, astrocytosis, neuronal loss and neural tissue from affected individuals contains an infectious agent, the prion, setting these diseases apart from other neurodegenerative diseases. Prion replication is thought to involve in essence the self-perpetuating conversion of the host-encoded cellular prion protein (PrP C ) into a misfolded form (PrP Sc ) that tends to aggregate and may be neurotoxic (for reviews [1,157]). Prion replication may also occur in lymphoid tissues but is there poorly if not pathogenic (for review [126], [133]). PrP C is a protein with two variably occupied glycosylation sites. It is attached at the outer of the plasma membrane by a glycosylphosphatidylinositol anchor. Its secondary structure is rich in alpha-helix and the protein is likely to be in a monomeric state in mild detergents. While its precise physiological function has not been assigned, PrP C is essential for prion replication and neurotoxicity to occur [57,129]. Upon infection, PrP C is refolded -without apparent post-translational modification -into beta-sheet -rich PrP Sc , initially in the presence of exogenous PrP Sc and then by an autocatalytic process. It leads to the formation of aggregates, sometimes of amyloid type, that can be differentiated from PrP C because of their partial resistance to protease digestion and of their insolubility into non-denaturing detergents [153]. Proteinase K, the most commonly used protease, di...

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Section: 'Absolute' Species Barriermentioning

confidence: 99%

mentioning

confidence: 99%

Prion agent diversity and species barrier

2008

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“…Browning et al developed the first transgenic mouse expressing cervid PrP [10], which was subsequently utilized as a bioassay to detect CWD prions in muscle [2]. Several transgenic mice expressing cervid PrP have since been produced using different constructs and sequences [10,41,44,52,84] (detailed in Tab.…”

Section: Tools For Cwd Research: Cell Lines and Cervid Prp Expressingmentioning

confidence: 99%

“…Several transgenic mice expressing cervid PrP have since been produced using different constructs and sequences [10,41,44,52,84] (detailed in Tab. I).…”

Section: Tools For Cwd Research: Cell Lines and Cervid Prp Expressingmentioning

confidence: 99%

“…The widespread occurrence of CWD in farmed and free-ranging cervids has led to a surge in CWD research, focused on understanding species susceptibility, transmission and pathogenesis, spatial epidemiology, diagnostic tools, strains, and cervid PrP structure. Transgenic mice expressing cervid PrP have been generated in five laboratories [10,41,44,52,84], providing useful tools for CWD research. In this review, the latest advances in CWD research are discussed.…”

Section: Introductionmentioning

confidence: 99%

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A prion disease of cervids: Chronic wasting disease

2008

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-Chronic wasting disease (CWD) is a prion disease of deer, elk, and moose, initially recognized in Colorado mule deer. The discovery of CWD beyond the borders of Colorado and Wyoming, in Canada and as far east as New York, has led to its emergence as a prion disease of international importance. Epidemiological studies indicate that CWD is horizontally transmitted among free-ranging animals, potentially indirectly by prion-containing secreta or excreta contaminating the environment. Experimental CWD transmission attempts to other wild and domestic mammals and to transgenic mice expressing the prion protein of cattle, sheep, and humans have shed light on CWD species barriers. Transgenic mice expressing the cervid prion protein have proven useful for assessing the genetic influences of Prnp polymorphisms on CWD susceptibility. Accumulating evidence of CWD pathogenesis indicates that the misfolded prion protein or prion infectivity seems to be widely disseminated in many nonneural organs and in blood. This review highlights contemporary research findings in this prion disease of free-ranging wildlife.

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Assistance Current Effect for Prevention of Voltage Reversal in Stacked Microbial Fuel Cell Systems

et al. 2015

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Herein, an assistance current is suggested as a new approach to prevent voltage reversal in series‐connected microbial fuel cells (MFCs). This approach can be used to revive reversed MFCs, as a supporting assistance current is applied through an assistance anode when voltage reversal occurs. However, when the assistance current is applied without control over the assistance current, voltage reversal is regenerated. Therefore, to control the assistance current, the suggested approach is complemented by the application of variable resistors between the anode and assistance electrode. As a result, the regenerated voltage reversal is restored and all voltages of MFC units in the stacked MFC remain positive. Thus, it is expected that this approach could resolve voltage reversal that occurs in series‐connected MFCs, and thus increase the applicability of MFC technology to the production of practically usable electricity, with potential for extension to large‐scale MFCs.

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Transmission of Prions from Mule Deer and Elk with Chronic Wasting Disease to Transgenic Mice Expressing Cervid PrP

Cited by 180 publications

References 44 publications

Prion agent diversity and species barrier

Prion agent diversity and species barrier

A prion disease of cervids: Chronic wasting disease

Assistance Current Effect for Prevention of Voltage Reversal in Stacked Microbial Fuel Cell Systems

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