Publications

the nature of the unconventional slow infection agents remains a puzzle.	unconventional slow infections are progressive transmissible degenerative disorders of the central nervous system. the human diseases belonging to this group are creutzfeld-jakob disease, kuru, and gerstmann-straussler syndrome. scrapie, transmissible mink encephalopathy, chronic wasting disease of mule deer and elk, and the recently discovered bovine spongiform encephalopathy are similar diseases found in animals. unusual characteristics of the unconventional slow infections clearly distinguish ...	1989	2568119
spongiform encephalopathies in cervidae.	the known host range of naturally-occurring transmissible spongiform encephalopathies has expanded in recent years to include wild ruminants. chronic wasting disease (cwd) occurs in mule deer (odocoileus hemionus hemionus) and rocky mountain elk (cervus elaphus nelsoni) in colorado and wyoming, united states of america. these species belong to the family cervidae. cases have occurred primarily in captive animals but a few affected free-ranging animals have been identified. clinical disease in bo ...	1992	1617203
[spongiform encephalopathies with special reference to bovine spongiform encephalopathy].	in switzerland bovine spongiform encephalopathy (bse) was detected for the first time in november 1990. it is a transmissible disease of the central nervous system similar to creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss) and kuru in man, and, in animals, scrapie in sheep and goats, chronic wasting disease (cwd) in captive mule deer and elk of north america and transmissible mink encephalopathy (tme) of farm reared mink. the infectious agent of the spongiform ence ...	1992	1615298
ultrastructural neuropathology of chronic wasting disease in captive mule deer.	chronic wasting disease (cwd), a progressive and uniformly fatal neurological disorder, is characterized neuropathologically by intraneuronal vacuolation, spongiform change of the neuropil and astrocytic hyperplasia and hypertrophy. ultrastructural neuropathological findings consist of (1) extensive vacuolation in neuronal processes, within myelin sheaths, formed by splitting at the major dense lines or within axons; (2) dystrophic neurites (dendrites, axonal preterminals and myelinated axons co ...	1993	8480515
neuropathology of chronic wasting disease of mule deer (odocoileus hemionus) and elk (cervus elaphus nelsoni).	the pathology of the central nervous system of nine mule deer (odocoileus hemionus) and six elk (cervus elaphus nelsoni) with chronic wasting disease, a spongiform encephalopathy of mule deer and elk, was studied by light microscopy. lesions were similar in both species and were characterized by spongiform transformation of gray matter, intracytoplasmic vacuolation of neurons, neuronal degeneration and loss, astrocytic hypertrophy and hyperplasia, occurrence of amyloid plaques, and absence of si ...	1993	8442326
the biology and molecular biology of scrapie-like diseases.	the transmissible spongiform encephalopathies (tse's) are degenerative diseases of the central nervous system which naturally affect man (creutzfeldt-jakob disease [cjd], gerstmann-sträussler syndrome [gss], kuru), sheep and goats (scrapie), cattle (bovine spongiform encephalopathy [bse]), mink (transmissible mink encephalopathy), mule deer, elk and antelope (chronic wasting disease). spongiform encephalopathies have also been diagnosed in captive species of zoo antelope and in domestic cats. mu ...	1993	8219804
electron microscopic findings in brain of rocky mountain elk with chronic wasting disease.	we report here the electron microscopic findings in brain of rocky mountain elk with chronic wasting disease (cdw), a progressive and fatal neurological disorder of wild ruminants characterized neuropathologically by intraneuronal vacuolation, spongiform change of the neuropil and astrocytic hypertrophy and hyperplasia. ultrastructural findings included membrane-bound vacuoles in neuronal elements, increased number of glial filaments, dystrophic neurites, numerous neuritic plaques, hirano bodies ...	1994	7982031
bovine spongiform encephalopathy and new variant creutzfeldt-jakob disease.	bovine spongiform encephalopathy (bse) and creutzfeldt-jakob disease (cjd) belong to a group of degenerative neurological disorders collectively known as the transmissible spongiform encephalopathies (tses). the group also includes scrapie of sheep and goats, kuru of humans, chronic wasting disease of mule deer and elk and transmissible encephalopathy of mink. these fatal diseases cause behavioural changes, alterations of sensation, changes in mental state and ataxia. the typical pathology is no ...	1998	10326298
the host range of chronic wasting disease is altered on passage in ferrets.	chronic wasting disease (cwd), a member of the transmissible spongiform encephalopathies (tses), was first identified in captive mule and black-tail deer in 1967. due to the failure to transmit cwd to rodents, we investigated the use of ferrets (mustela putorius furo) as a small animal model of cwd. the inoculation of cwd into ferrets resulted in an incubation period of 17-21 months on primary passage that shortened to 5 months by the third ferret passage. the brain tissue of animals inoculated ...	1998	9837794
[transmissible spongiform encephalopathies in animals].	transmissible spongiform encephalopathies in animals are known for centuries. in particular scrapie in sheep and goats occurs worldwide; it spreads as a natural disease and is genetically controlled. chronic wasting disease (cwd) in the united states (wyoming and colorado) also spreads as natural disease among free ranging and captive elk and mule deer. in contrast, transmissible mink encephalopathy (tme) of mink in fur producing farms is caused by contaminated feed; the source of this food cont ...	1998	9611347
preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle.	to determine the transmissibility of chronic wasting disease (cwd) to cattle and to provide information about clinical course, lesions, and suitability of currently used diagnostic procedures for detection of cwd in cattle, 13 calves were inoculated intracerebrally with brain suspension from mule deer naturally affected with cwd. between 24 and 27 months postinoculation, 3 animals became recumbent and were euthanized. gross necropsies revealed emaciation in 2 animals and a large pulmonary absces ...	2001	11243374
molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies.	the transmissible spongiform encephalopathy (tse) diseases are a group of rare, fatal, and transmissible neurodegenerative diseases that include kuru and creutzfeldt-jakob disease (cjd) in humans, scrapie in sheep, transmissible mink encephalopathy (tme), and chronic wasting disease (cwd) in mule deer and elk. over the last 20 yr, they have gone from a fascinating but relatively obscure group of diseases to one that is a major agricultural and economic problem as well as a threat to human health ...	2004	15156065
chronic wasting disease of cervids.	chronic wasting disease (cwd) has recently emerged in north america as an important prion disease of captive and free-ranging cervids (species in the deer family). cwd is the only recognized transmissible spongiform encephalopathy (tse) affecting free-ranging species. three cervid species, mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni), are the only known natural hosts of cwd. endemic cwd is well established in southern wyomin ...	2004	15148993
chronic wasting disease.	chronic wasting disease (cwd) is a unique transmissible spongiform encephalopathy (tse) of mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni). the natural history of cwd is incompletely understood, but it differs from scrapie and bovine spongiform encephalopathy (bse) by virtue of its occurrence in nondomestic and free-ranging species. cwd has many features in common with scrapie, including early widespread distribution of disease ...	2005	16145200
experimental second passage of chronic wasting disease (cwd(mule deer)) agent to cattle.	to compare clinicopathological findings in first and second passage chronic wasting disease (cwd(mule deer)) in cattle, six calves were inoculated intracerebrally with brain tissue derived from a first-passage cwd-affected calf in an earlier experiment. two uninoculated calves served as controls. the inoculated animals began to lose both appetite and weight 10-12 months later, and five subsequently developed clinical signs of central nervous system (cns) abnormality. by 16.5 months, all cattle h ...	2006	16423572
susceptibility of cattle to first-passage intracerebral inoculation with chronic wasting disease agent from white-tailed deer.	fourteen, 3-month-old calves were intracerebrally inoculated with the agent of chronic wasting disease (cwd) from white-tailed deer (cwdwtd) to compare the clinical signs and neuropathologic findings with those of certain other transmissible spongiform encephalopathies (tse, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie, cwd of mule deer [cwdmd], bovine spongiform encephalopathy [bse], and transmissible mink encephalopathy). two uninoculated cal ...	2007	17606510
chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein.	chronic wasting disease (cwd) is a prion disease that affects free-ranging and captive cervids, including mule deer, white-tailed deer, rocky mountain elk and moose. cwd-infected cervids have been reported in 14 usa states, two canadian provinces and in south korea. the possibility of a zoonotic transmission of cwd prions via diet is of particular concern in north america where hunting of cervids is a popular sport. to investigate the potential public health risks posed by cwd prions, we have in ...	2010	20610667
rapid detection of cwd prp: comparison of tests designed for the detection of bse or scrapie.	chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) mainly affecting cervids in north america. the accumulation of an abnormal form of host-encoded prion protein (prp(cwd) ) in the cns and lymphoid tissues is characteristic of the disease and known to be caused by pathogenic prion proteins (prp(res) ), which are thought to be transmitted mainly by contact with body fluids, such like saliva. species known to be naturally infected by cwd include rocky mountain elk (cer ...	2011	22212828
functional genomics approach for identification of molecular processes underlying neurodegenerative disorders in prion diseases.	prion diseases or transmissible spongiform encephalopathies (tses) are infectious neurodegenerative disorders leading to death. these include cresutzfeldt-jakob disease (cjd), familial, sporadic and variant cjd and kuru in humans; and animal tses include scrapie in sheep, bovine spongiform encephalopathy (bse) in cattle, chronic wasting disease (cwd) of mule deer and elk, and transmissible mink encephalopathy. all these tses share common pathological features such as accumulation of mis-folded p ...	2012	23372423
historical overview of prion diseases: a view from afar.	the transmissible spongiform encephalopathies (tses), or prion diseases, are a group of neurodegenerative disorders which include kuru, creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker (gss) syndrome, and fatal familial insomnia in men, natural scrapie in sheep, goats and mufflons, transmissible mink encephalopathy in ranch-reared mink, chronic wasting disease of mule deer and elk, bovine spongiform encephalopathy or "mad cow disease" and its analogues in several exotic species of ...	2012	22505359
molecular mechanisms of chronic wasting disease prion propagation.	prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. examples include kuru, once the leading cause of death among the fore people in papua new guinea and caused by mortuary feasting; bovine spongiform encephalopathy (bse) and its subsequent transmission to humans in the form of variant creutzfeldt-jakob disease (vcjd), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. the ...	2017	28193766