Chronic Wasting Disease

A transmissible spongiform encephalopathy (prion disease) of DEER and elk characterized by chronic weight loss leading to death. It is thought to spread by direct contact between animals or through environmental contamination with the prion protein (PRIONS).

Also Known As:

Networked: 325 relevant articles (5 outcomes, 19 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Scrapie
2.	Bovine Spongiform Encephalopathy (Mad Cow Disease)
3.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)
4.	Progressive Supranuclear Palsy (Steele Richardson Olszewski Syndrome)
5.	Sporadic Creutzfeldt-Jakob Disease

Experts

1.	Hoover, Edward A: 41 articles (12/2021 - 10/2001)
2.	Telling, Glenn C: 36 articles (01/2022 - 12/2004)
3.	Mathiason, Candace K: 29 articles (12/2021 - 10/2006)
4.	Miller, Michael W: 24 articles (12/2021 - 10/2001)
5.	Balachandran, Aru: 17 articles (01/2018 - 05/2004)
6.	Spraker, Terry R: 17 articles (01/2016 - 10/2001)
7.	Zabel, Mark D: 16 articles (01/2022 - 09/2007)
8.	O'Rourke, Katherine I: 14 articles (11/2017 - 07/2003)
9.	Denkers, Nathaniel D: 13 articles (12/2020 - 06/2010)
10.	Bartz, Jason C: 12 articles (06/2022 - 11/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chronic Wasting Disease:

1.	PrionsIBA 07/01/2013 - "Could avian scavengers translocate infectious prions to disease-free areas initiating new foci of chronic wasting disease?" 04/01/2023 - "In light of the increasing importance of chronic wasting disease, the application of mouse transgenesis to study this burgeoning and highly contagious prion disorder, in particular recent insights derived from gene-targeting approaches, will be a major focus of this review." 01/01/2021 - "IMPORTANCE This study reports the first finding of detectable levels of prions linked to chronic wasting disease in a tick collected from a clinically infected elk. " 12/08/2020 - "Studies in bank voles reveal strain differences between chronic wasting disease prions from Norway and North America." 01/01/2017 - "In nature, cervids are infected with chronic wasting disease (CWD) prions by oral and nasal mucosal exposure, and studies of early CWD pathogenesis have implicated pharyngeal lymphoid tissue as the earliest sites of prion accumulation. "
2.	Prion ProteinsIBA 06/16/2023 - "A previous study reported that the M132L single nucleotide polymorphism (SNP) of the prion protein gene (PRNP) is associated with susceptibility to chronic wasting disease (CWD) in elk. " 01/22/2019 - "Transmission studies of chronic wasting disease to transgenic mice overexpressing human prion protein using the RT-QuIC assay." 07/01/2015 - "The purpose of our study was to describe the progressive accumulation of the abnormal conformer of the prion protein (PrP(CWD)) and spongiform degeneration in a single section of brain stem in Rocky Mountain elk (Cervus elaphus nelsoni) with chronic wasting disease (CWD). " 10/01/2023 - "Chronic Wasting Disease (CWD), associated with misfolded prion proteins of cervids, serves as an ideal model for evaluating new RT-QuIC methodologies. " 09/08/2023 - "Heterogeneity of pathological prion protein accumulation in the brain of moose (Alces alces) from Norway, Sweden and Finland with chronic wasting disease."
3.	Codon (Codons)IBA 01/01/2022 - "However, 20 chronic wasting disease-positive white-tailed deer possessing codon 96SS genotypes were also observed, including one that was lymph node and obex positive. " 01/01/2021 - "New and distinct chronic wasting disease strains associated with cervid polymorphism at codon 116 of the Prnp gene." 03/09/2018 - "Pathologic and biochemical characterization of PrPSc from elk with PRNP polymorphisms at codon 132 after experimental infection with the chronic wasting disease agent." 01/01/2017 - "Chronic Wasting Disease (CWD) is a prion disease of cervids regarded as a public health problem in North America, and polymorphisms at specific codons in the PRNP gene are associated with this disease. " 09/01/2012 - "Diagnostic accuracy of rectal mucosa biopsy testing for chronic wasting disease within white-tailed deer (Odocoileus virginianus) herds in North America: effects of age, sex, polymorphism at PRNP codon 96, and disease progression."
4.	Methionine (L-Methionine)FDA Link 12/03/2007 - "A methionine/leucine polymorphism at the corresponding site in the Rocky Mountain elk PRNP gene is associated with variation in relative susceptibility and incubation time in the cervid TSE chronic wasting disease. " 03/01/2013 - "In order to assess the susceptibility of bank voles to chronic wasting disease (CWD), we inoculated voles carrying isoleucine or methionine at codon 109 (Bv109I and Bv109M, respectively) with CWD isolates from elk, mule deer and white-tailed deer. " 02/01/2008 - "The elk prion protein gene (PRNP) encodes either methionine (M) or leucine (L) at codon 132, the L132 allele apparently affording protection against chronic wasting disease (CWD). "
5.	Leucine (L-Leucine)FDA Link 12/03/2007 - "A methionine/leucine polymorphism at the corresponding site in the Rocky Mountain elk PRNP gene is associated with variation in relative susceptibility and incubation time in the cervid TSE chronic wasting disease. " 02/01/2008 - "The elk prion protein gene (PRNP) encodes either methionine (M) or leucine (L) at codon 132, the L132 allele apparently affording protection against chronic wasting disease (CWD). "
6.	SaponinsIBA 01/01/2012 - "Here, we demonstrate that PMCAb with saponin dramatically increases the sensitivity of detection for chronic wasting disease (CWD) agent without compromising the specificity of the assay (i.e., no false positive results). "
7.	Proteins (Proteins, Gene)FDA Link 03/13/2021 - "This study assessed deiminated protein and EV profile signatures in plasma from sixteen healthy wild female reindeer, collected in Iceland during screening for parasites and chronic wasting disease. " 05/01/2012 - "Sensitivity of protein misfolding cyclic amplification versus immunohistochemistry in ante-mortem detection of chronic wasting disease." 01/01/2012 - "Highly efficient amplification of chronic wasting disease agent by protein misfolding cyclic amplification with beads (PMCAb)." 01/01/2019 - "Remarkably, these infectious proteins have been responsible for widespread disease epidemics, including kuru in humans, bovine spongiform encephalopathy in cattle, and chronic wasting disease in cervids, the latter of which has spread across North America and recently appeared in Norway and Finland. " 12/01/2016 - "Chronic wasting disease (CWD) in cervids and bovine spongiform encephalopathy (BSE) in cattle are prion diseases that are caused by the same protein-misfolding mechanism, but they appear to pose different risks to humans. "
8.	Amino AcidsFDA Link 01/01/2023 - "Our previous studies using gene-targeted mouse models of chronic wasting disease (CWD) demonstrated that Norway and North America cervids are infected with distinct prion strains that respond differently to naturally occurring amino acid variation at residue 226 of the prion protein. " 10/01/2020 - "Chronic wasting disease (CWD) is caused by an unknown spectrum of prions and has become enzootic in populations of cervid species that express cellular prion protein (PrPC) molecules varying in amino acid composition. " 01/15/2014 - "We and others have studied how specific amino acid residue differences between species impact prion conversion and have found that the β2-α2 loop region of the mouse prion protein (residues 165-175) markedly influences infection by sheep scrapie, BSE, mouse-adapted scrapie, deer chronic wasting disease, and hamster-adapted scrapie prions. " 08/15/2007 - "To study the pathogenesis of chronic wasting disease (CWD) in deer and elk, transgenic (tg) mice were generated that expressed the prion protein (PrP) of deer containing a glycine at amino acid (aa) 96 and a serine at aa 225 under transcriptional control of the murine PrP promoter. "
9.	Peptide Hydrolases (Proteases)FDA Link 09/01/2021 - "Moreover, we reveal that TC-5RW is able to directly decrease protease-resistant PrPSc and inhibit the seeding activity of PrPSc from chronic wasting disease and various human prion diseases. " 02/01/2010 - "Experimental oral transmission of chronic wasting disease to red deer (Cervus elaphus elaphus): early detection and late stage distribution of protease-resistant prion protein." 09/01/2007 - "Chronic wasting disease (CWD) of cervids is associated with conversion of the normal cervid prion protein, PrP(C), to a protease-resistant conformer, PrP(CWD). " 07/01/2006 - "Chronic wasting disease (CWD) is a fatal, emerging disease of cervids associated with transmissible protease-resistant prion proteins. " 01/01/2006 - "Inhibition of protease-resistant prion protein formation in a transformed deer cell line infected with chronic wasting disease."
10.	Endopeptidase K (Proteinase K)IBA 01/01/2015 - "Unlike scrapie and chronic wasting disease there is no cell culture model allowing the replication of proteinase K resistant BSE (PrPBSE) and the further in vitro study of this disease. " 01/01/2018 - "Abbreviations: PrP, prion protein; PK, proteinase K; ShIC, shaking-induced conversion; RENAGE, resolution enhanced native acidic gel electrophoresis; TEM, transmission electron microscopy; TUDCA, taurourspdeoxycholic acid; BSE, bovine spongiform encephalopathy; CWD, chronic wasting disease; CJD, Creutzfeldt Jakob disease; GSS, Gerstmann-Sträussler-Scheinker syndrome; FFI, fatal familial insomnia; PrPc, cellular prion protein; recPrPC, recombinant monomeric prion protein; PrPSc, infectious particle of misfolded prion protein; RT-QuIC, real-time quaking-induced conversion; PMCA, Protein Misfolding Cyclic Amplification; LPS, lipopolysaccharide; EGCG, epigallocatechin gallate; GN8, 2-pyrrolidin-1-yl-N-[4-[4-(2-pyrrolidin-1-yl-acetylamino)-benzyl]-phenyl]-acetamide; DMSO, dimethyl sulfoxide; ScN2A, scrapie infected neuroblastoma cells; IC50, inhibitory concentration for 50% reduction; recMoPrP 23-231, recombinant full-length mouse prion protein residues 23-231; EDTA; PICUP, photo-induced cross-linking of unmodified protein; BSA, bovine serum albumin;; PMSF, phenylmethanesulfonyl fluoride."

Therapies and Procedures

1.	Therapeutics 04/01/2012 - "Assessment of prospective preventive therapies for chronic wasting disease in mule deer." 04/01/2012 - "Non-toxic preventative therapies are also needed, with ongoing cases of Bovine Spongiform Encephalopathy (BSE) and the spread of Chronic Wasting Disease (CWD) being a growing concern. " 02/01/2009 - "There is increasing pressure to find effective decontaminants for blood supplies, as variant Creutzfeldt Jakob Disease (vCJD) has been shown to be transmissible by blood, and to find non-toxic preventative therapies, with ongoing cases of Bovine Spongiform Encephalopathy (BSE) and the spread of Chronic Wasting Disease (CWD). "
2.	Surgical Instruments (Clip) 05/01/2004 - "For the assessment of risk scenarios originating from these findings (e.g., alimentary transmission of pathogens associated with bovine spongiform encephalopathy [BSE] and chronic wasting disease [CWD] via tainted beef and game or iatrogenic dissemination of CJD agent through contaminated surgical instruments) more detailed information about the time course of PrP(Sc) accumulation in muscles at preclinical and clinical stages of incubation is needed. "
3.	Lasers (Laser) 11/02/2017 - "Electron microscopic and confocal laser microscopy analysis of amyloid plaques in chronic wasting disease transmitted to transgenic mice."
4.	Blood Transfusion (Blood Transfusions) 12/01/2020 - "Successful transmission of the chronic wasting disease (CWD) agent to white-tailed deer by intravenous blood transfusion."