Creutzfeldt-Jakob Disease: A Case-Control Study

Bobowick, A R; Brody, Jacob A.; M, Matthews; Roos, R.; Gajdusek, D. Carleton

doi:10.1093/oxfordjournals.aje.a121567

Cited by 77 publications

(31 citation statements)

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“…How prions causing disease arise in patients with sporadic forms is unknown; hypotheses include horizontal transmission of prions from humans or animals (242), somatic mutation of the PrP gene, and spontaneous conversion of PrP C into PrP Sc (5,15). Because numerous attempts to establish an infectious link between sporadic CJD and a preexisting prion disease in animals or humans have been unrewarding, it seems unlikely that transmission features in the pathogenesis of sporadic prion disease (9)(10)(11)(12)243).…”

mentioning

confidence: 99%

Molecular characterization of proteolytic cleavage sites of the Pseudomonas syringae effector AvrRpt2

Chisholm

Dahlbeck

Krishnamurthy

et al. 2005

Proc. Natl. Acad. Sci. U.S.A.

137

138

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Prions are unprecedented infectious pathogens that cause a group of invariably fatal neurodegenerative diseases by an entirely novel mechanism. Prion diseases may present as genetic, infectious, or sporadic disorders, all of which involve modification of the prion protein (PrP). Bovine spongiform encephalopathy (BSE), scrapie of sheep, and Creutzfeldt-Jakob disease (CJD) of humans are among the most notable prion diseases. Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrP Sc ). The normal, cellular PrP (PrP C ) is converted into PrP Sc through a posttranslational process during which it acquires a high ␤-sheet content. The species of a particular prion is encoded by the sequence of the chromosomal PrP gene of the mammals in which it last replicated. In contrast to pathogens carrying a nucleic acid genome, prions appear to encipher strain-specific properties in the tertiary structure of PrP Sc . Transgenetic studies argue that PrP Sc acts as a template upon which PrP C is refolded into a nascent PrP Sc molecule through a process facilitated by another protein. Miniprions generated in transgenic mice expressing PrP, in which nearly half of the residues were deleted, exhibit unique biological properties and should facilitate structural studies of PrP Sc . While knowledge about prions has profound implications for studies of the structural plasticity of proteins, investigations of prion diseases suggest that new strategies for the prevention and treatment of these disorders may also find application in the more common degenerative diseases.

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mentioning

confidence: 99%

Molecular characterization of proteolytic cleavage sites of the Pseudomonas syringae effector AvrRpt2

Chisholm

Dahlbeck

Krishnamurthy

et al. 2005

Proc. Natl. Acad. Sci. U.S.A.

137

138

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“…The duration of the human illness varies from patient to patient (22), some lasting one to several months (type I), others less than 9 months (type II), and still others lasting as long as 1-2 years (type III). The present investigation in hamsters strongly suggests that some of these Creutzfeldt-Jakob subgroups, displaying different syndromes and different durations of human disease, may be related to the action of different strains of the Creutzfeldt-Jakob agent.…”

Section: Resultsmentioning

confidence: 99%

Interspecies transmission of Creutzfeldt-Jakob disease to Syrian hamsters with reference to clinical syndromes and strains of agent.

Manuelidis¹,

Gorgacz²,

Manuelidis³

1978

Proc. Natl. Acad. Sci. U.S.A.

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Experimental Creutzfeldt-Jakob disease was serially transmitted from guinea pigs to Syrian hamsters with 100% incidence, morbidity, and mortality. All animals developed a subacute spongiform virus encephalopathy with neuronal destruction and concomitant astrocytic changes. In the first passage three different clinical syndromes were recorded, each with widely variant incubation times; these results suggested there may be different strains of the Creutzfeldt-Jakob agent, some of which may be partially separated when the agent is passaged from one species to another. Accumulations of neurofilaments in neuronal perikarya and processes and increased lipofuscin were suggestive ofchanges seen insenility and aging.The "subacute spongiform virus encephalopathies" (1), characterized by degeneration of the central nervous system with neuronal loss, sponginess of the tissue (status spongiosus), and astrocytosis without accompanying inflammatory infiltrates, have been traced to unconventional replicating agents. In naturally occurring diseases these agents replicate in species ranging from human beings (kuru and Creutzfeldt-Jakob disease) to mink (transmissible mink encephalopathy) and sheep (scrapie). Because the exact nature of the agents comprising this group is unknown, the relationship between agents isolated from the naturally occurring diseases in different species and propagated in different experimental hosts forms the only basis for their comparison. Serial transmission of Creutzfeldt-Jakob disease of human beings to guinea pigs (2, 3), and more recently to mice (4), has been reported from this laboratory. The present paper reports the serial transmission of experimental Creutzfeldt-Jakob disease from guinea pigs to hamsters. Variations in the incubation periods, clinical syndromes, and histological lesions were observed. These results suggested the existence of different strains of the Creutzfeldt-Jakob agent.Experimental data reported in interspecies transmission of experimental scrapie are compared. MATERIALS AND METHODSThree young golden hamsters weighing 25-35 g were inoculated with 0.05 ml intracerebrally and 0.1 ml intraperitoneally of a 1:10 suspension of brain in physiological saline from two guinea pigs that had developed Creutzfeldt-Jakob disease during the fifth passage of the disease. Both guinea pigs used for inoculation revealed on histological examination the characteristic features of a spongiform virus encephalopathy.For serial transmission, seven healthy hamsters (second passage) were injected with similarly prepared inoculum from a sick hamster of the first passage in the same quantities and routes of inoculation as that used in the first passage. When the hamster of the second passage developed the experimental disease (Table 1), a third group of six hamsters was similarly inoculated. From one sick hamster of the third passage the infection was passaged to six healthy hamsters, and the fourth passage is presently in progress. Routinely, half the brain of the sick hamster was frozen to...

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“…Einen Überblick über die bisherigen Studien geben die Tabellen 15 und 16. Die Studienergebnisse von Bobowick et al (1973), Kondo und Kuroiwa (1982), Davanipour et al (1985), Harries-Jones et al (1988) (Poser et al 1999, Heinemann et al 2007a (Davanipour et al 1985). …”

Section: Anzahlen Von Operationen Und Bluttransfusionenunclassified