The neuropathology of kuru and variant Creutzfeldt–Jakob disease

McLean, Catriona

doi:10.1098/rstb.2008.0086

Cited by 13 publications

(10 citation statements)

References 14 publications

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“…It has been noted that kuru shows considerable similarities to sCJD VV2 or sCJD MV 2K (14,53,100,101,117). Using archival tissues from transmission experiments, Parchi et al recently provided evidence that strengthened the theory that kuru originated from cannibalism of an individual with sCJD VV2 or MV 2 K, as no indication of MM1 prions could be found, even in subjects carrying the MM or MV genotype that had succumbed to kuru (119).…”

Section: Kurumentioning

confidence: 99%

Prion Disease: A Tale of Folds and Strains

Kretzschmar¹,

Tatzelt²

2013

Brain Pathology

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Research on prions, the infectious agents of devastating neurological diseases in humans and animals, has been in the forefront of developing the concept of protein aggregation diseases. Prion diseases are distinguished from other neurodegenerative diseases by three peculiarities. First, prion diseases, in addition to being sporadic or genetic like all other neurodegenerative diseases, are infectious diseases. Animal models were developed early on (a long time before the advent of transgenic technology), and this has made possible the discovery of the prion protein as the infectious agent. Second, human prion diseases have true equivalents in animals, such as scrapie, which has been the subject of experimental research for many years. Variant Creutzfeldt-Jakob disease (vCJD) is a zoonosis caused by bovine spongiform encephalopathy (BSE) prions. Third, they show a wide variety of phenotypes in humans and animals, much wider than the variants of any other sporadic or genetic neurodegenerative disease. It has now become firmly established that particular PrP(Sc) isoforms are closely related to specific human prion strains. The variety of human prion diseases, still an enigma in its own right, is a focus of this article. Recently, a series of experiments has shown that the concept of aberrant protein folding and templating, first developed for prions, may apply to a variety of neurodegenerative diseases. In the wake of these discoveries, the term prion has come to be used for Aβ, α-synuclein, tau and possibly others. The self-propagation of alternative conformations seems to be the common denominator of these "prions," which in future, in order to avoid confusion, may have to be specified either as "neurodegenerative prions" or "infectious prions."

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Section: Kurumentioning

confidence: 99%

Prion Disease: A Tale of Folds and Strains

Kretzschmar¹,

Tatzelt²

2013

Brain Pathology

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“…To this end, a few papers re-evaluated historic material has been published [ 137 ]: We [ 138 ] studied by PrP-immunohistochemistry the case of a young male kuru victim of the name Kupenota from the South Fore region whose brain tissue had transmitted disease to chimpanzees, and McLean et al . [ 139 , 140 ] examined a series of 11 archived cases of kuru. In contrast to the classical studies described above, both papers stressed the presence of typical spongiform change present in deep layers (III–V) of the cingulate, occipital, enthorrinal and insular cortices, and in the subiculum.…”

Section: Neuropathologymentioning

confidence: 99%

“…[ 147 ] classification or type 2 CJD of the Parchi et al . [ 148 ] classification [ 139 ]. Of note, immunocytochemistry with 12F10 antibodies revealed a stronger signal than that using 3F4 anti-PrP antibodies [ 139 ].…”

Section: Neuropathologymentioning

confidence: 99%

Kuru: A Journey Back in Time from Papua New Guinea to the Neanderthals’ Extinction

Liberski

2013

Pathogens

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Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923. In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Strä ussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.

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“…We (64) and others (75–77) studied kuru using modern PrP immunohistochemistry. We were privileged to examine a case of a young male kuru patient named “K” from the South Fore region whose brain tissue had transmitted the disease to chimpanzees, and McLean et al (76, 77) examined a series of 11 cases of kuru. In contrast to the classical studies described above, both articles stressed the presence of typical spongiform change present, as in sporadic CJD, in deep cortical layers (III–V) of the cingulate, entorhinal and insular cortices, and in the subiculum.…”

Section: Introductionmentioning

confidence: 99%

“…In contrast to the classical studies described above, both articles stressed the presence of typical spongiform change present, as in sporadic CJD, in deep cortical layers (III–V) of the cingulate, entorhinal and insular cortices, and in the subiculum. The occipital cortex is variably affected (77). Spongiform change was also observed in the putamen and caudate, and some putaminal neurons contained intraneuronal vacuoles.…”

Section: Introductionmentioning

confidence: 99%

Kuru

Liberski

Sikorska

Lindenbaum

et al. 2012

Journal of Neuropathology & Experimental Neurology

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Kuru was the first human transmissible spongiform encephalopathy (TSE) or prion disease identified, occurring in the Fore linguistic group of Papua New Guinea. Kuru was a uniformly fatal cerebellar ataxic syndrome, usually followed by choreiform and athetoid movements. Kuru imposed a strong balancing selection on the Fore population, with individuals homozygous for the 129 Met allele of the gene (PRNP) encoding for prion protein (PrP) being the most susceptible. The decline in the incidence of kuru in the Fore has been attributed to the exhaustion of the susceptible genotype and ultimately by discontinuation of exposure via cannibalism. Neuropathologically, kuru-affected brains were characterized by widespread degeneration of neurons, astroglial and microglial proliferation, and the presence of amyloid plaques. These early findings have been confirmed and extended by recent immunohistochemical studies for the detection of the TSE-specific PrP (PrPTSE). Confocal laser microscopy also showed the concentration of glial fibrillary acidic protein–positive astrocytic processes at the plaque periphery. The fine structure of plaques corresponds to that described earlier by light microscopy. The successful experimental transmission of kuru led to the awareness of its similarity to Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease and formed a background against which the recent epidemics of iatrogenic and variant Creutzfeldt-Jakob disease could be studied.

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The neuropathology of kuru and variant Creutzfeldt–Jakob disease

Cited by 13 publications

References 14 publications

Prion Disease: A Tale of Folds and Strains

Prion Disease: A Tale of Folds and Strains

Kuru: A Journey Back in Time from Papua New Guinea to the Neanderthals’ Extinction

Kuru

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