Chapter title |
Creutzfeldt-Jakob disease.
|
---|---|
Chapter number | 6 |
Book title |
Neurodegenerative Diseases
|
Published in |
Advances in experimental medicine and biology, March 2012
|
DOI | 10.1007/978-1-4614-0653-2_6 |
Pubmed ID | |
Book ISBNs |
978-1-4614-0652-5, 978-1-4614-0653-2
|
Authors |
Sikorska B, Knight R, Ironside JW, Liberski PP, Sikorska, Beata, Knight, Richard, Ironside, James W., Liberski, Paweł P., Beata Sikorska, Richard Knight, James W. Ironside, Paweł P. Liberski |
Abstract |
Creutzfeldt-Jakob disease (CJD), a neurodegenerative disorder that is the commonest form of human prion disease or transmissible spongiform encephalopathies (TSEs). Four types of CJD are known: Sporadic (sCJD), familial or genetic (gCJD); iatrogenic (iCJD) and variant CJD (vCJD). The latter results from transmission of bovine spongiform encephalopathy (BSE) from cattle to humans. The combination of PrP(Sc) peptide (either 21 kDa or 19 kDa) and the status of the codon 129 of the gene (PRNP) encoding for PrP (either Methionine or Valine) is used to classify sCJD into 6 types: MM1 and MV1, the most common; VV2; MV2 (Brownell/Oppenheimer syndrome); MM2; VV1 and sporadic fatal insomnia, in that order of prevalence. Genetic CJD is caused by diverse mutations in the PRNP gene. The neuropathology of CJD consists of spongiform change, astro- and microgliosis and poorly defined neuronal loss. In a proportion of cases, amyloid plaques, like those of kuru, are seen. PrP immunohistochemistry reveals different types of PrP(Sc) deposits - the most common is the synaptic-type, but perivacuolar, perineuronal and plaque-like deposits may be also detected. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Korea, Republic of | 1 | 100% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 2 | 2% |
Spain | 1 | 1% |
Ecuador | 1 | 1% |
France | 1 | 1% |
Unknown | 85 | 94% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 13 | 14% |
Student > Bachelor | 13 | 14% |
Researcher | 9 | 10% |
Student > Postgraduate | 4 | 4% |
Student > Master | 4 | 4% |
Other | 12 | 13% |
Unknown | 35 | 39% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 14 | 16% |
Biochemistry, Genetics and Molecular Biology | 12 | 13% |
Agricultural and Biological Sciences | 11 | 12% |
Neuroscience | 9 | 10% |
Chemistry | 2 | 2% |
Other | 6 | 7% |
Unknown | 36 | 40% |