Inhibition of Protease-Resistant Prion Protein Formation in a Transformed Deer Cell Line Infected with Chronic Wasting Disease

Raymond, Gregory J.; Olsen, Emily A.; Lee, Kil Sun; Raymond, Lynne D.; Bryant, Patricia; Baron, Gerald S.; Caughey, Winslow S.; Kocisko, David A.; McHolland, Linda E.; Favara, Cynthia; Langeveld, J.P.M.; Zijderveld, F.G. van; Mayer, Richard T.; Miller, Michael W.; Williams, Elizabeth; Caughey, Byron

doi:10.1128/jvi.80.2.596-604.2006

Cited by 69 publications

(68 citation statements)

References 43 publications

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“…A CWD-susceptible cell line derived from cervid brain fibroblasts has been used to screen inhibitors of CWD infection, for example, pentosan polysulfate [69]. This CWD specific assay may identify compounds that inhibit CWD propagation.…”

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

confidence: 99%

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

confidence: 99%

A prion disease of cervids: Chronic wasting disease

2008

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“…Deer Chronic wasting disease [116] murine prion strains. L929 mouse fibroblasts propagate 22L, ME7, and RML while MG20 microglial cultures can be infected by ME7 and Chandler strains and by the mouse-adapted BSE agent.…”

Section: Fibroblast-likementioning

confidence: 99%

“…These cell models have distinct and complementary features that proved useful to study various aspects of sheep scrapie infection. More recently, a chronic wasting disease isolate was successfully transmitted to a mule deer-derived fibroblast cell line [116]. Although multiplication of the human prion was reported on one occasion [73], there is no available cell model permissive to human Creutzfeldt-Jakob disease (CJD) or cattle BSE agents.…”

Section: Cell Models Propagating Naturally-occurring Prion Isolatesmentioning

confidence: 99%

“…A variety of these molecules were tested in infected N2a cultures and some showed inhibitory activity on abnormal PrP accumulation [30]. Another porphyrin compound was subsequently found to be active in the MDB fibroblast-like cell line infected with a CWD isolate [116]. The most efficient molecules were tested in several animal models, including tg7 transgenic mice infected with hamster prions [69,112] and mice infected with the RML strain of the murine prion [113].…”

Section: Tetrapyrrole Compoundsmentioning

confidence: 99%

“…not previously adapted to rodents) to cultured cells, and this also provided evidence that PrP res can be readily detected in infected cells from the non-neuronal lineage. Since then, prion multiplication was demonstrated in several other non-neuronal cell systems, including fibroblasts [116,147], muscle cells [39], microglial cells [61], and another natural isolate (from chronic wasting disease) was successfully transmitted to a mule deer cell line [116].…”

Section: Introductionmentioning

confidence: 99%

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Cell models of prion infection

Vilette

2007

Vet. Res.

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-Due to recent renewal of interest and concerns in prion diseases, a number of cell systems permissive to prion multiplication have been generated in the last years. These include established cell lines, neuronal stem cells and primary neuronal cultures. While most of these models are permissive to experimental, mouse-adapted strains of prions, the propagation of natural field isolates from sheep scrapie and chronic wasting disease has been recently achieved. These models have improved our knowledge on the molecular and cellular events controlling the conversion of the PrP C protein into abnormal isoforms and on the cell-to-cell spreading of prions. Infected cultured cells will also facilitate investigations on the molecular basis of strain identity and on the mechanisms that lead to neurodegeneration. The ongoing development of new cell models with improved characteristics will certainly be useful for a number of unanswered critical issues in the prion field.

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