Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-669899f699-7tmb6 Total loading time: 0 Render date: 2025-05-04T13:40:23.917Z Has data issue: false hasContentIssue false

9 - Prion diseases

from Section 1 - Agents

Published online by Cambridge University Press:  12 January 2010

By
Marc L. Turner ,
Patricia E. Hewitt ,
Moira Bruce ,
James W. Ironside ,
David J. Anstee  and
Gary Mallinson
Edited by
John A. J. Barbara ,
Fiona A. M. Regan  and
Marcela Contreras
Marc L. Turner
Affiliation:
Professor of Cellular Therapy, University of Edinburgh; Clinical Director/Consultant Haematologist, Edinburgh and S.E. Scotland Blood Transfusion Centre, Royal Infirmary of Edinburgh, Edinburgh, Scotland
Patricia E. Hewitt
Affiliation:
Consultant Specialist in Transfusion Microbiology, NHS Blood and Transplant Colindale, London, UK
Moira Bruce
Affiliation:
Institute for Animal Health Neuropathogenesis Unit, Edinburgh, UK
James W. Ironside
Affiliation:
Professor of Clinical Neuropathology, National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK
David J. Anstee
Affiliation:
Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK
Gary Mallinson
Affiliation:
Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK
John A. J. Barbara
Affiliation:
University of the West of England, Bristol
Fiona A. M. Regan
Affiliation:
HNSBT and Hammersmith Hospitals NHS Trust, London
Marcela Contreras
Affiliation:
University of the West of England, Bristol
Get access

Summary

Introduction

Prion diseases include a spectrum of disorders in animals and man (see Table 9.1). Scrapie, endemic in sheep and goat populations throughout most of the world, was first recognized over 250 years ago and was demonstrated to be experimentally transmissible in 1936. Chronic wasting disease (CWD) is endemic in Rocky Mountain elk, white-tailed deer and mule deer in several areas of the USA and is increasing in both incidence and geographic distribution. The routes by which these two endemic prion diseases are transmitted remain unclear. Transmissible mink encephalopathy was first recorded to have occurred in 1947 in farmed mink in Wisconsin and was probably transmitted through prion infected food.

Bovine spongiform encephalopathy (BSE) was first recognized in the UK in 1985/86 (Wells et al., 1987). Affected cattle become apprehensive, hypersensitive, ataxic and generally difficult to handle, giving rise to the common name of mad cow disease. It remains unclear whether BSE arose spontaneously in cattle or resulted from transmission of scrapie from sheep, but onward transmission is thought to have occurred through the practice of feeding cattle ruminant-derived meat and bone meal. Over 180,000 clinical cases of BSE have been reported in the UK since 1985, though the annual incidence has now fallen to just over 100 cases per annum. Over 4500 infected cattle have been detected elsewhere, mainly in Europe, the majority associated with the export of BSE infected cattle or meat and bone meal from the UK. It is estimated that between 1 and 2 million cattle may have become infected and entered the human food chain before developing evidence of clinical disease (Donnelly et al. 2002).

Type
Chapter
Information
Transfusion Microbiology , pp. 141 - 152
Publisher: Cambridge University Press
Print publication year: 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Book purchase

Temporarily unavailable

References

Brown, P., Cervenakova, L. and Diringer, H. (2001) Blood infectivity and the prospects for a diagnostic screening test in Creutzfeldt-Jakob disease. J. Lab Clin. Med, 137 (1), 5–13.CrossRefGoogle ScholarPubMed
European Commission (EC) (2006) Regulation No 253/2006 amendment to Annexe X of EC Regulation 999/2001, Chapter C point 4.
Grosset, A., Moskowitz, K., Nelsen, C., et al. (2005) Rapid presymptomatic detection of PrPSc via conformationally responsive palindromic PrP peptides. Peptides, 26 (11), 2193–200.CrossRefGoogle ScholarPubMed
Houston, F., Foster, J. D., Chong, A., et al. (2000) Transmission of BSE by blood transfusion in sheep. Lancet, 356 (9234), 999–1000.CrossRefGoogle Scholar
HPA (2006) http://www.hpa.org.uk/hpa/news/articles/press_releases/2006/060209_cjd.htm
Jones, M., Peden, A. H., Prowse, C. V., et al. (2007) In vitro amplification and detection of variant Creutzfeldt-Jacob disease PrPScJ. Pathol, 213, 21–6.CrossRefGoogle ScholarPubMed
Korth, C., Stierli, B., Streit, P., et al. (1997) Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature, 390 (6655), 74–7.CrossRefGoogle ScholarPubMed
Llewelyn, C. A., Hewitt, P. E., Knight, R. S., et al. (2004) Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet, 363 (9407), 417–21.CrossRefGoogle ScholarPubMed
Miele, G., Manson, J. and Clinton, M. (2001) A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nat. Med, 7 (3), 361–4.CrossRefGoogle ScholarPubMed
Peden, A. H., Head, M. W., Ritchie, D. L., et al. (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet, 364 (9433), 527–9.CrossRefGoogle Scholar
Peretz, D., Williamson, R. A., Kaneko, K., et al. (2001) Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature, 412 (6848), 739–43.CrossRefGoogle ScholarPubMed
Saa, P., Castilla, J. and Soto, C. (2006) Presymptomatic detection of prions in blood. Science, 313 (5783), 92–4.CrossRefGoogle ScholarPubMed
Saborio, G. P., Permanne, B. and Soto, C. (2001) Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature, 411 (6839), 810–3.CrossRefGoogle ScholarPubMed
Silveira, J. R., Raymond, G. J., Hughson, A. G., et al. (2005) The most infectious prion protein particles. Nature, 437 (7056), 257–61.CrossRefGoogle ScholarPubMed
Soto, C., Anderes, L., Suardi, S.et al. (2005) Pre-symptomatic detection of prions by cyclic amplification of protein misfolding. FEBS Lett, 579 (3), 638–42.CrossRefGoogle ScholarPubMed
Will, R. G., Ironside, J. W., Zeidler, M., et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet, 347 (2006), 921–5.CrossRefGoogle ScholarPubMed
Barclay, G. R., Hope, J., Barnard, G., et al. (1999) Distribution of cell associated prion protein in normal adult blood determined by flow cytometry. Br J Hematol, 107, 804–14.CrossRefGoogle ScholarPubMed
Barclay, G. R., Houston, F., Halliday, S., et al. (2002) Comparative analysis of normal prion protein expression on human, rodent and ruminant blood cells using a panel of anti-prion antibodies. Transfusion, 42, 517–26.CrossRefGoogle Scholar
Brown, K. L., Stewart, K., Ritchie, D. L., et al. (1999a) Scrapie replication in lymphoid tissues depends on prion protein-expressing follicular dendritic cells. Nat Med, 5, 1308–12.CrossRefGoogle Scholar
Brown, P. (2000) BSE and transmission through blood. Lancet, 356, 955–6.CrossRefGoogle ScholarPubMed
Brown, P., Cervenakova, L. and Diringer, H. (2001) Blood infectivity and the prospects for a diagnostic screening test in Creutzfeldt-Jakob disease. J Lab Clin Med, 137, 5–13.CrossRefGoogle ScholarPubMed
Brown, P., Cervenakova, L., McShane, L. M., et al. (1999b) Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt-Jakob disease in humans. Transfusion, 39, 1169–78.CrossRefGoogle Scholar
Brown, P., Preece, M. A., and Will, R. G. (1992) ‘Friendly fire’ in medicine: hormones, homografts and Creutzfeldt-Jakob disease. Lancet, 340, 24–7.CrossRefGoogle Scholar
Brown, P., Rohwer, R. G., Dunstan, B. C., et al. (1998) The distribution of infectivity in blood components and plasma derivatives in experimental models of transmissible spongiform encephalopathy, Transfusion, 38, 810–6.CrossRefGoogle ScholarPubMed
Bruce, M., Brown, K. L., Mabbott, N. A., et al. (2000) Follicular dendritic cells in TSE pathogenesis. Immunol Today, 21, 442–6.CrossRefGoogle ScholarPubMed
Bruce, M. E., McConnell, I., Will, R. G., et al. (2001) Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet, 358, 208–9.CrossRefGoogle ScholarPubMed
Bruce, M. E., Will, R. G., Ironside, J. W., et al. (1997) Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature, 389, 498–501.CrossRefGoogle ScholarPubMed
Collinge, J., Sidle, K. C. L., Meads, J., et al. (1996) Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature, 383, 685–90.CrossRefGoogle ScholarPubMed
Creutzfeldt, H. G. (1920) Uber eine eigenartige herdfomige Erkrankung des Zentralnervensystems. Zeitschrift fur die gesamte Neurologie und Psychiatrie, 57, 1–18.CrossRefGoogle Scholar
Donnelly, C. A., Ferguson, N. M., Ghani, A. C., et al. (2002) Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels. Proc R Soc Lond B, 269, 2179–90.CrossRefGoogle ScholarPubMed
Foster, P. R. (2000) Prions and blood products. Ann Med, 32, 501–13.CrossRefGoogle ScholarPubMed
Gajdusek, D. C. and Zigas, V. (1957) Degenerative disease of the central nervous system in New Guinea: epidemic of ‘kuru’ in the native population. N Eng J Med, 257, 974–8.CrossRefGoogle Scholar
Ghani, A. C., Donnelly, C. A., Ferguson, N. M., et al. (2003) Updated projections of future vCJD deaths in the UK. BMC Infectious Diseases, 3, 8.CrossRefGoogle ScholarPubMed
Gregori, L, McCombie, N., Palmer, D., et al. (2004) Effectiveness of leucoreduction for removal of infectivity of transmissible spongiform encephalopathies from blood. Lancet, 364, 529–31.CrossRefGoogle ScholarPubMed
Head, M. W., Ritchie, D., Smith, N., et al. (2004) Peripheral tissue involvement in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative and biochemical study. Am J Pathol, 164, 143–53.CrossRefGoogle ScholarPubMed
Hilton, D. A., Fathers, E., Edwards, P., et al. (2004b) Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet, 352, 703–4.CrossRefGoogle Scholar
Hilton, D. A., Ghani, A. C., Conyers, L., et al. (2002) Accumulation of prion protein in tonsil and appendix: review of tissue samples. BMJ, 325, 633–4.CrossRefGoogle ScholarPubMed
Hilton, D. A., Ghani, A. C., Conyers, L., et al. (2004a) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol, 203, 733–9.CrossRefGoogle Scholar
Houston, F., Foster, J. D., Chong, A., et al. (2000) Transmission of BSE by blood transfusion in sheep. Lancet, 356, 999–1000.CrossRefGoogle Scholar
Jakob, A. (1921) Uber eine der multiplen Sklerose klinisch nahestehende Erkrankung des Zentralnervensystems (spastische Pseudosklerose) mit bemerkenswertem anatomischem Befunde. Mitteilung eines vierten Falles. Med Klin, 17, 372–6.Google Scholar
Klein, M. A., Frigg, R., Flechsig, E., et al. (1997) A crucial role for B-cells in neuroinvasive scrapie. Nature, 390, 687–90.CrossRefGoogle ScholarPubMed
Llewelyn, C. A., Hewitt, P. E., Knight, R. S., et al. (2004) Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet, 363, 417–21.CrossRefGoogle ScholarPubMed
Mabbott, N. A. and Bruce, M. E. (2001) The immunobiology of TSE diseases. J Gen Virol, 82, 2307–18.CrossRefGoogle ScholarPubMed
Mabbott, N. A., Bruce, M. E., Botto, M., et al. (2001) Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie. Nat Med, 7, 485–7.CrossRefGoogle ScholarPubMed
Mabbott, N. A., Mackay, F., Minns, F., et al. (2000) Temporary inactivation of follicular dendritic cells delays neuroinvasion of scrapie. Nat Med, 6, 719–20.CrossRefGoogle ScholarPubMed
MacGregor, I. (2001) Prion protein and developments in its detection. Transfusion, 11, 3–14.CrossRefGoogle ScholarPubMed
MacGregor, I., Hope, J., Barnard, G., et al. (1999) The distribution of normal prion protein in human blood. Vox Sang, 77, 88–96.CrossRefGoogle Scholar
McBride, P. A., Eikelenboom, P., Kraal, G., et al. (1992) PrP protein is associated with follicular dendritic cells of spleens and lymph nodes in uninfected and scrapie-infected mice. J Pathol, 168, 413–8.CrossRefGoogle ScholarPubMed
McBride, P. A., Schultz-Schaeffer, W. J., Donaldson, M., et al. (2001) Early spread of scrapie from the gastrointestinal tract to the central nervous system involves autonomic fibres of the splanchnic and vagus nerves. J Virol, 75, 9320–27.CrossRefGoogle Scholar
Miele, G., Manson, J. and Clinton, M. (2001) A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nat Medicine, 7, 361–4.CrossRefGoogle ScholarPubMed
National CJD Surveillance Unit, available at: www.cjd.ed.ac.uk/figures.htm, accessed 23 October 2007.
Palmer, M. S., Dryden, A. J., Hughes, J. T., et al. (1991) Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature, 352, 340–2.CrossRefGoogle ScholarPubMed
Peden, A. H., Head, M. W., Ritchie, D. L., et al. (2004) Preclinical vCJD infection after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet, 364 (9433), 527–9.CrossRefGoogle Scholar
Prusiner, S. B. (1982) Novel proteinaceous infectious particles cause scrapie. Science, 216, 136–44.CrossRefGoogle ScholarPubMed
Rohwer, R. G. (2000) Titer, distribution and transmissibility of blood-borne TSE infectivity. Cambridge Healthtech Institute 6th Annual Meeting ‘Blood Product Safety: TSE, Perception versus Reality’. Maclean, Virginia, USA.
Turner, M. L. and Ironside, J. W. (1998) New-variant Creutzfeldt-Jakob disease: the risk of transmission by blood transfusion. Blood Rev, 12, 255–68.CrossRefGoogle ScholarPubMed
Wadsworth, J. D. F., Joiner, S., Hill, A. F., et al. (2001) Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet, 358, 171–80.CrossRefGoogle ScholarPubMed
Wells, G. A. H., Scott, A. C., Johnson, C. T., et al. (1987) A novel progressive spongiform encephalopathy in cattle. Vet Rec, 121, 419–20.CrossRefGoogle ScholarPubMed
Will, R. G. (2003) Acquired prion disease: iatrogenic CJD, variant CJD, kuru. B Med Bull, 66, 255–65.CrossRefGoogle ScholarPubMed
Will, R. G., Ironside, J. W., Zeidler, M., et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet, 347, 921–5.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

  • Prion diseases
    • By Marc L. Turner, Professor of Cellular Therapy, University of Edinburgh; Clinical Director/Consultant Haematologist, Edinburgh and S.E. Scotland Blood Transfusion Centre, Royal Infirmary of Edinburgh, Edinburgh, Scotland, Patricia E. Hewitt, Consultant Specialist in Transfusion Microbiology, NHS Blood and Transplant Colindale, London, UK, Moira Bruce, Institute for Animal Health Neuropathogenesis Unit, Edinburgh, UK, James W. Ironside, Professor of Clinical Neuropathology, National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK, David J. Anstee, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK, Gary Mallinson, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK
  • Edited by John A. J. Barbara, University of the West of England, Bristol, Fiona A. M. Regan, Marcela Contreras, University of the West of England, Bristol
  • Book: Transfusion Microbiology
  • Online publication: 12 January 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545245.013
Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

  • Prion diseases
    • By Marc L. Turner, Professor of Cellular Therapy, University of Edinburgh; Clinical Director/Consultant Haematologist, Edinburgh and S.E. Scotland Blood Transfusion Centre, Royal Infirmary of Edinburgh, Edinburgh, Scotland, Patricia E. Hewitt, Consultant Specialist in Transfusion Microbiology, NHS Blood and Transplant Colindale, London, UK, Moira Bruce, Institute for Animal Health Neuropathogenesis Unit, Edinburgh, UK, James W. Ironside, Professor of Clinical Neuropathology, National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK, David J. Anstee, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK, Gary Mallinson, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK
  • Edited by John A. J. Barbara, University of the West of England, Bristol, Fiona A. M. Regan, Marcela Contreras, University of the West of England, Bristol
  • Book: Transfusion Microbiology
  • Online publication: 12 January 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545245.013
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Prion diseases
    • By Marc L. Turner, Professor of Cellular Therapy, University of Edinburgh; Clinical Director/Consultant Haematologist, Edinburgh and S.E. Scotland Blood Transfusion Centre, Royal Infirmary of Edinburgh, Edinburgh, Scotland, Patricia E. Hewitt, Consultant Specialist in Transfusion Microbiology, NHS Blood and Transplant Colindale, London, UK, Moira Bruce, Institute for Animal Health Neuropathogenesis Unit, Edinburgh, UK, James W. Ironside, Professor of Clinical Neuropathology, National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK, David J. Anstee, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK, Gary Mallinson, Bristol Institute for Transfusion Sciences, NHS Blood and Transplant, Bristol, UK
  • Edited by John A. J. Barbara, University of the West of England, Bristol, Fiona A. M. Regan, Marcela Contreras, University of the West of England, Bristol
  • Book: Transfusion Microbiology
  • Online publication: 12 January 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511545245.013
Available formats
×