Prion protein accumulation in the spinal cords of patients with sporadic and growth hormone associated Creutzfeldt-Jakob disease
Abstract
An immunohistological study of the spinal cord in 20 cases of sporadic and 4 iatrogenic (growth hormone) cases of Creutzfeldt-Jakob (CJD) disease patients was performed to detect the presence of disease specific prion protein using a number of different antisera. Prion protein was present in all the growth hormone recipients and in 11 of the 20 sporadic CJD cases. Plaque-like deposits of prion protein were found in all the growth hormone cases and three of the sporadic cases. This is the first demonstration of the topographic immunolocalisation of prion protein in the spinal cord of CJD patients, a feature which could help elucidate some important aspects of the pathogenesis of CJD.
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The location of PrPTSE in the spinal cord substantia gelatinosa in kuru is reminiscent of that typical of iatrogenic CJD cases after peripheral inoculation.
“The latter location was reminiscent of that typical of iatrogenic CJD cases after peripheral inoculation (78).”
From Kuru (2012) · cites this paper as [78]
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Kuru, the First Human Prion Disease
Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019) · Viruses · doi:10.3390/v11030232 · Cited by 69 · see citing works
Liberski, P. P., Sikorska, B., Lindenbaum, S., Goldfarb, L. G., McLean, C., Hainfellner, J. A., & Brown, P. (2012) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3182444efd · Cited by 50 · see citing works
- Authors
- Goodbrand, I. A., Ironside, J. W., Nicolson, D., & Bell, J. E.
- Affiliations
- Western General Hospital (GB)
- Corresponding author
- Goodbrand, I.A.
- ORCID
- Ironside, J.W.
- Venue
- Neuroscience Letters
- Publisher
- Elsevier BV
- Published
- 1995-01
- Volume
- 183
- Issue
- 1-2
- Pages
- 127-130
- ISSN
- 0304-3940 (ISSN-L)
- OpenAlex
- W2067243437
- Cited by
- 47
- References
- 0
- Citations by year
- 2025: 1 · 2020: 2 · 2019: 1 · 2017: 2 · 2013: 2 · 2012: 4
- FWCI
- 1.36
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neuroscience Letters (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- prion protein, Creutzfeldt-Jakob disease, spinal cord, iatrogenic CJD, immunolocalization, prion pathogenesis
- MeSH terms
- Antibodies, Autopsy, Humans, Immunohistochemistry, Creutzfeldt-Jakob Syndrome, Prions, Growth Hormone, Spinal Cord
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