James W Ironside

Summary

Affiliation: University of Edinburgh
Country: UK

Publications

  1. ncbi request reprint BSE and vCJD cause disturbance to uric acid levels
    Tamuna Lekishvili
    Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath, BA2 7AY, UK
    Exp Neurol 190:233-44. 2004
  2. ncbi request reprint Review: Creutzfeldt-Jakob disease
    J W Ironside
    Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
    Brain Pathol 6:379-88. 1996
  3. pmc Best Practice No 172: pituitary gland pathology
    J W Ironside
    Division of Pathology, School of Clinical and Molecular Medicine, University of Edinburgh, Western General Hospital, Edingurgh EH4 2XU, UK
    J Clin Pathol 56:561-8. 2003
  4. ncbi request reprint Pathological diagnosis of variant Creutzfeldt-Jakob disease
    J W Ironside
    Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
    APMIS 110:79-87. 2002
  5. ncbi request reprint Neuropathology and molecular biology of variant Creutzfeldt-Jakob disease
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Department of Pathology, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, UK
    Curr Top Microbiol Immunol 284:133-59. 2004
  6. ncbi request reprint Neuropathology of variant Creutzfeldt-Jakob disease
    James W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom
    Acta Neurobiol Exp (Wars) 62:175-82. 2002
  7. ncbi request reprint Neuropathology of variant Creutzfeldt-Jakob disease
    James W Ironside
    National CJD Surveillance Unit, Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, United Kingdom
    C R Biol 325:27-31. 2002
  8. ncbi request reprint Laboratory diagnosis of variant Creutzfeldt-Jakob disease
    J W Ironside
    Departments of Pathology, Clinical Neurosciences, CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Histopathology 37:1-9. 2000
  9. ncbi request reprint Variant Creutzfeldt-Jakob disease and its transmission by blood
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, UK
    J Thromb Haemost 1:1479-86. 2003
  10. doi request reprint Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay
    Michael Jones
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, The Roslin Institute, Edinburgh, UK
    Neuroreport 19:1783-6. 2008

Detail Information

Publications73

  1. ncbi request reprint BSE and vCJD cause disturbance to uric acid levels
    Tamuna Lekishvili
    Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath, BA2 7AY, UK
    Exp Neurol 190:233-44. 2004
    ..These findings suggest that changes in uric acid may aid differential diagnosis of vCJD. Uric acid could be used to inhibit cell death or PrP(Sc) formation in prion disease...
  2. ncbi request reprint Review: Creutzfeldt-Jakob disease
    J W Ironside
    Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
    Brain Pathol 6:379-88. 1996
    ....
  3. pmc Best Practice No 172: pituitary gland pathology
    J W Ironside
    Division of Pathology, School of Clinical and Molecular Medicine, University of Edinburgh, Western General Hospital, Edingurgh EH4 2XU, UK
    J Clin Pathol 56:561-8. 2003
    ....
  4. ncbi request reprint Pathological diagnosis of variant Creutzfeldt-Jakob disease
    J W Ironside
    Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
    APMIS 110:79-87. 2002
    ....
  5. ncbi request reprint Neuropathology and molecular biology of variant Creutzfeldt-Jakob disease
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Department of Pathology, Western General Hospital, University of Edinburgh, Edinburgh EH4 2XU, UK
    Curr Top Microbiol Immunol 284:133-59. 2004
    ..Histological, genetic and biochemical techniques are essential tools for the adequate diagnosis and investigation of human prion diseases...
  6. ncbi request reprint Neuropathology of variant Creutzfeldt-Jakob disease
    James W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom
    Acta Neurobiol Exp (Wars) 62:175-82. 2002
    ....
  7. ncbi request reprint Neuropathology of variant Creutzfeldt-Jakob disease
    James W Ironside
    National CJD Surveillance Unit, Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, United Kingdom
    C R Biol 325:27-31. 2002
    ..Continued surveillance is required to investigate this possibility in the UK and other countries where BSE has been reported...
  8. ncbi request reprint Laboratory diagnosis of variant Creutzfeldt-Jakob disease
    J W Ironside
    Departments of Pathology, Clinical Neurosciences, CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Histopathology 37:1-9. 2000
    ..Continued surveillance is required to further investigate this possibility, with the need to investigate autopsy tissues from suspected cases by histological and biochemical techniques...
  9. ncbi request reprint Variant Creutzfeldt-Jakob disease and its transmission by blood
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, UK
    J Thromb Haemost 1:1479-86. 2003
    ....
  10. doi request reprint Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay
    Michael Jones
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, The Roslin Institute, Edinburgh, UK
    Neuroreport 19:1783-6. 2008
    ....
  11. doi request reprint An antibody to the aggregated synthetic prion protein peptide (PrP106-126) selectively recognizes disease-associated prion protein (PrP) from human brain specimens
    Michael Jones
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Brain Pathol 19:293-302. 2009
    ..These results suggest that P1:1 recognizes an epitope formed during the structural rearrangement or aggregation of the PrP that is common to the major PrP(Sc) types found in the most common forms of human prion disease...
  12. doi request reprint Transmissions of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice
    Diane L Ritchie
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK
    J Gen Virol 90:3075-82. 2009
    ..Overall, the results closely resembled those of previously reported transmissions of BSE in the same mouse strains, consistent with BSE being the origin of all of these vCJD cases...
  13. pmc Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease
    Matthew T Bishop
    National Creutzfeldt Jakob Disease Research and Surveillance Unit, University of Edinburgh, Bryan Matthews Building, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK
    Brain 136:1139-45. 2013
    ..These studies demonstrate that spleen tissue from a PRNP MV genotype individual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious agent can be present in the spleen without CNS involvement...
  14. doi request reprint Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species
    Young Pyo Choi
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Edinburgh, UK
    Brain Pathol 21:298-307. 2011
    ....
  15. pmc Molecular model of prion transmission to humans
    Michael Jones
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, Scotland EH4 2XU, UK
    Emerg Infect Dis 15:2013-6. 2009
    ..Protein misfolding cyclic amplification showed that conformation of PrPd partly determines host susceptibility...
  16. ncbi request reprint Sporadic Creutzfeldt-Jakob disease: further twists and turns in a convoluted protein
    Mark W Head
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2 XU, UK
    Brain 129:2238-40. 2006
  17. ncbi request reprint Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002
    Mark W Head
    National Creutzfeldt Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Edinburgh, United Kingdom
    Ann Neurol 55:851-9. 2004
    ..The results show PrP(Sc) in variant Creutzfeldt-Jakob disease to be remarkably stereotyped. In contrast, considerable heterogeneity in PrP(Sc) exists both between and within cases of sporadic Creutzfeldt-Jakob disease...
  18. pmc No major change in vCJD agent strain after secondary transmission via blood transfusion
    Matthew T Bishop
    National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    PLoS ONE 3:e2878. 2008
    ..Since there are likely to be carriers of the vCJD agent in the general population, there is a potential for further infection by routes such as blood transfusion or contaminated surgical instruments...
  19. ncbi request reprint Review: pathology of variant Creutzfeldt-Jakob disease
    Alexander H Peden
    National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, United Kingdom
    Folia Neuropathol 42:85-91. 2004
    ..Continuing surveillance of vCJD in the UK and other countries in which BSE has been identified will be necessary for future estimations of disease numbers worldwide...
  20. doi request reprint Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease
    Michael Jones
    University of Edinburgh, Western General Hospital, UK
    Transfusion 49:376-84. 2009
    ....
  21. pmc Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study
    James W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU corrected
    BMJ 332:1186-8. 2006
    ..To perform prion protein gene (PRNP) codon 129 analysis in DNA extracted from appendix tissue samples that had tested positive for disease associated prion protein...
  22. pmc Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay
    Young Pyo Choi
    National CJD Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK
    J Virol 84:12030-8. 2010
    ..These results suggest that conformational stability represents a further dimension to a complete description of potentially phenotype-related properties of PrP(Sc) in human prion diseases...
  23. ncbi request reprint Abnormal prion protein in the pituitary in sporadic and variant Creutzfeldt-Jakob disease
    Alexander H Peden
    National Creutzfeldt Jakob Disease Surveillance Unit NCJDSU and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK
    J Gen Virol 88:1068-72. 2007
    ....
  24. pmc Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease
    Helen M Yull
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Edinburgh, EH4 2XU, UK
    Am J Pathol 168:151-7. 2006
    ..These results indicate that PrP(Sc) molecular typing is based on quantitative rather than qualitative phenomena and point to a complex relationship between prion protein biochemistry, disease phenotype and agent strain...
  25. pmc Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative, and biochemical study
    Mark W Head
    National Creutzfeldt Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    Am J Pathol 164:143-53. 2004
    ..The results show that involvement of the lymphoreticular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isoform of PrP(Sc) found is influenced by the cell type in which it accumulates...
  26. ncbi request reprint Risks of transmission of variant Creutzfeldt-Jakob disease by blood transfusion
    Alexander H Peden
    National Kreutzfeld Jakob Disiease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, EH4 2XU, United Kingdom
    Folia Neuropathol 43:271-8. 2005
    ....
  27. ncbi request reprint The spectrum of safety: variant Creutzfeldt-Jakob disease in the United Kingdom
    James W Ironside
    Department of Clinical Neuropathology, The University of Edinburgh, and the National CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland
    Semin Hematol 40:16-22. 2003
    ..Because it is impossible to detect presymptomatic individuals who carry the etiologic prion agent, the United Kingdom has adopted measures to secure the safety of its blood supply and to minimize potential routes of infection...
  28. doi request reprint The application of in vitro cell-free conversion systems to human prion diseases
    Michael Jones
    Components and vCJD Research, National Science Laboratories, Scottish National Blood Transfusion Service, Edinburgh, EH 17 7QT, UK
    Acta Neuropathol 121:135-43. 2011
    ....
  29. doi request reprint Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008
    Mark W Head
    National Creutzfeldt Jakob Disease Research and Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh EH4 2XU, UK
    Brain 136:1102-15. 2013
    ....
  30. pmc Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversion
    Alexander H Peden
    National CJD Research and Surveillance unit, School of Molecular and Clinical Medicine, University of Edinburgh, Edinburgh, UK
    J Gen Virol 93:438-49. 2012
    ..Comparison of these QuIC results with those previously obtained using PMCA suggested that these two seemingly similar assays differ in important respects...
  31. pmc Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease
    Alexander H Peden
    National Creutzfeldt Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom
    Am J Pathol 168:927-35. 2006
    ....
  32. doi request reprint Human embryonic stem cells rapidly take up and then clear exogenous human and animal prions in vitro
    Zuzana Krejciova
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Western General Hospital, Edinburgh, UK
    J Pathol 223:635-45. 2011
    ....
  33. ncbi request reprint Mad cows and monkey business: the end of vCJD?
    Mark W Head
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    Lancet 365:730-1. 2005
  34. ncbi request reprint Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient
    Alexander H Peden
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    Lancet 364:527-9. 2004
    ..These findings have major implications for future estimates and surveillance of vCJD in the UK...
  35. doi request reprint Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob diseased brain using conformation-dependent immunoassay and Western blot
    Young Pyo Choi
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Edinburgh, UK
    J Gen Virol 92:727-32. 2011
    ..Additionally, the combination of protease digestion and CDI detection demonstrated, for the first time, the presence of PrP(Sc) in variant CJD brains that is susceptible to proteolysis under standard conditions...
  36. ncbi request reprint Molecular pathology in neurodegenerative diseases
    Alexander H Peden
    National CJD Research and Surveillance unit, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Curr Drug Targets 13:1548-59. 2012
    ..The concept of proteinopathy as a common mechanism in neurodegenerative disorders is explored, and we highlight the improvements in diagnosis and management required to improve our treatment of these devastating conditions...
  37. doi request reprint Further characterisation of the prion protein molecular types detectable in the NIBSC Creutzfeldt-Jakob disease brain reference materials
    Helen M Yull
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Edinburgh, United Kingdom
    Biologicals 37:210-5. 2009
    ..We conclude that these reagents provide a valuable resource for future research and development...
  38. pmc Prion protein accumulation and neuroprotection in hypoxic brain damage
    Neil F McLennan
    National Creutzfeldt Jakob Disease Surveillance Unit and Pathology Neuropathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, Scotland, UK
    Am J Pathol 165:227-35. 2004
    ..Furthermore, the infarct size in PrP-null mice was significantly greater than in the wild type, supporting the proposed role for PrP(C) in the neuroprotective adaptive cellular response to hypoxic injury...
  39. ncbi request reprint Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob disease
    Mark W Head
    National Creutzfeldt Jakob Disease Surveillance Unit and Department of Pathology, University of Edinburgh, Scotland, United Kingdom
    Invest Ophthalmol Vis Sci 44:342-6. 2003
    ..This study was conducted to assess the possible involvement of the eye in sporadic and variant CJD by testing for the presence of the disease-associated, protease-resistant isoform of the prion protein (PrP(Sc)) in ocular tissue...
  40. ncbi request reprint Application of an immunocapillary electrophoresis assay to the detection of abnormal prion protein in brain, spleen and blood specimens from patients with variant Creutzfeldt-Jakob disease
    Paula C Lourenco
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    J Gen Virol 87:3119-24. 2006
    ..In this context, the ICE assay was specific, but incompletely sensitive (55%). The method was unable to detect abnormal prion protein in variant CJD brain or spleen reference materials due to its loss during the extraction process...
  41. doi request reprint Management of a twenty-first century brain bank: experience in the BrainNet Europe consortium
    Jeanne E Bell
    Department of Pathology Neuropathology, University of Edinburgh, Wilkie Building, Teviot Place, Edinburgh, UK
    Acta Neuropathol 115:497-507. 2008
    ....
  42. doi request reprint Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease
    Craig A Heath
    Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK
    Ann Neurol 67:761-70. 2010
    ..Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue...
  43. pmc Application of Atomic Dielectric Resonance Spectroscopy for the screening of blood samples from patients with clinical variant and sporadic CJD
    Timothy J Fagge
    National CJD Surveillance Unit and Division of Pathology, University of Edinburgh School of Molecular and Clinical Medicine, Western General Hospital, Edinburgh EH4 2XU, UK
    J Transl Med 5:41. 2007
    ..Most assays aim to detect abnormal prion protein (PrPSc), although achieving required sensitivity is a challenge...
  44. pmc Differential protein profiling as a potential multi-marker approach for TSE diagnosis
    Janice B Barr
    The Roslin Institute and R D SVS, University of Edinburgh, Roslin, Midlothian, EH25 9PS, UK
    BMC Infect Dis 9:188. 2009
    ..Recently, concern that this marker may not be as reliable as previously thought, coupled with an urgentneed for a pre-clinical live animal test, has led to the search for alternative assays for the detection of TSE disease...
  45. doi request reprint The contribution of different prion protein types and host polymorphisms to clinicopathological variations in Creutzfeldt-Jakob disease
    Mark W Head
    The National CJD Research and Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Edinburgh, UK
    Rev Med Virol 22:214-29. 2012
    ....
  46. ncbi request reprint Variant Creutzfeldt-Jakob disease: an update
    James W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, EH4 2XU, United Kingdom
    Folia Neuropathol 50:50-6. 2012
    ..The recent report of a blood test that may be used to detect vCJD has raised the possibility of a new way to identify infected individuals, perhaps even before the onset of clinical symptoms...
  47. ncbi request reprint Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases
    Donald A Collie
    Department of Neuroradiology, Western General Hospital, Edinburgh, Scotland
    AJNR Am J Neuroradiol 24:1560-9. 2003
    ..This purpose of this larger study was to evaluate this sign prospectively and further define the MR imaging characteristics of vCJD...
  48. doi request reprint Endocrine mucin-producing sweat gland carcinoma: report of two cases of an under-recognized malignant neoplasm and review of the literature
    Catharine A Dhaliwal
    Department of Pathology, Western General Hospital, Edinburgh, United Kingdom
    Am J Dermatopathol 35:117-24. 2013
    ..The histological differential diagnosis is discussed and attention drawn to the role of immunohistochemistry in clarifying the nosological position of EMPSGC within the spectrum of cutaneous mucinous neoplasms...
  49. ncbi request reprint Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes
    Gabor G Kovacs
    Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases
    Brain Pathol 12:1-11. 2002
    ..Antibodies such as 6H4 and 12F10 failed to give this type of labeling and are therefore less likely to recognise non-pathological PrP material in immunohistochemistry...
  50. ncbi request reprint Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases
    Katsuya Satoh
    Department of Neurological Science, Tohoku University Graduate School of Medicine, 2 1 Seiryo machi, Sendai 980 8575, Japan
    J Gen Virol 84:2885-93. 2003
    ..The unique relationship of fPrP11-12 with CJD phenotype supports the view that the phenotypic heterogeneity of CJD is related to the formation of different types of disease-specific PrP and fragments thereof...
  51. pmc Accumulation of prion protein in tonsil and appendix: review of tissue samples
    David A Hilton
    Department of Histopathology, Derriford Hospital, Plymouth PL6 8DH
    BMJ 325:633-4. 2002
  52. ncbi request reprint Molecular classification of sporadic Creutzfeldt-Jakob disease
    Andrew F Hill
    MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK
    Brain 126:1333-46. 2003
    ..A novel PrP(Sc) type is presented, illustrating further heterogeneity in CJD, and suggesting that further molecular subtypes of CJD may exist at lower frequencies. A molecular classification of sporadic CJD is proposed...
  53. ncbi request reprint Correlative studies support lipid peroxidation is linked to PrP(res) propagation as an early primary pathogenic event in prion disease
    Marcus W Brazier
    Department of Pathology, The University of Melbourne, VIC 3010, Australia
    Brain Res Bull 68:346-54. 2006
    ....
  54. ncbi request reprint Laminar distribution of the pathological changes in the cerebral cortex in variant Creutzfeldt-Jakob disease (vCJD)
    Richard A Armstrong
    Vision Sciences, Aston University, Birmingham B4 7ET, UK
    Folia Neuropathol 40:165-71. 2002
    ..These patterns of laminar distribution suggest that the process of cortical degeneration may be distinctly different in vCJD compared with sCJD...
  55. pmc Disease-associated prion protein in vessel walls
    Oskar Koperek
    Institute of Neurology, University of Vienna, Waehringer Guertel 18 20, A 1097 Vienna, Austria
    Am J Pathol 161:1979-84. 2002
    ..We conclude that mobile cells in vessel walls like dendritic and monocyte/macrophage lineage cells may be involved in spread of disease-associated prion protein and possibly also of infectivity...
  56. pmc Tetracyclines affect prion infectivity
    Gianluigi Forloni
    Istituto di Ricerche Farmacologiche Mario Negri, Istituto Nazionale Neurologico Carlo Besta, and Istituto di Microbiologia e Immunologia Veterinaria, Universita degli Studi, 20100 Milano, Italy
    Proc Natl Acad Sci U S A 99:10849-54. 2002
    ..Our data suggest that these well characterized antibiotics reduce prion infectivity through a direct interaction with PrP(Sc) and are potentially useful for inactivation of BSE- or vCJD-contaminated products and prevention strategies...
  57. ncbi request reprint Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions
    Tetsuyuki Kitamoto
    Department of Neurological Science, Tohoku University Graduate School of Medicine, 2 1 Seiryo machi, Aoba ku, Sendai, Japan
    Biochem Biophys Res Commun 294:280-6. 2002
    ..Conversion of humanized PrP in the spleen provides a rapid and sensitive bioassay method to uncover the infectivity of human prions. This model should facilitate the prevention of infectious prion diseases...
  58. ncbi request reprint PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob Disease
    James E McCormack
    Neuropathogenesis Unit, Institute for Animal Health, West Mains Road, Edinburgh EH9 3JF, UK
    Gene 288:139-46. 2002
    ..Our data suggests that polymorphisms in the regulatory region of the PRNP gene may be a risk factor for CJD...
  59. ncbi request reprint Increased clusterin (apolipoprotein J) expression in human and mouse brains infected with transmissible spongiform encephalopathies
    Kensuke Sasaki
    Department of Neuropathology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
    Acta Neuropathol 103:199-208. 2002
    ....
  60. pmc Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions
    Yuzuru Taguchi
    Department of Neurological Science, Tohoku University Graduate School of Medicine, Sendai, Japan
    Am J Pathol 163:2585-93. 2003
    ..We compared the characteristic susceptibility profile of Ki-ChM with those of other precedent transgenic mice and discussed, including the prospects in developing genetically engineered mice susceptible to human prions...
  61. ncbi request reprint vCJD prion acquires altered virulence through trans-species infection
    Masahiro Asano
    Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, 2 1 Seiryo, Aoba, Sendai 980 8575, Japan
    Biochem Biophys Res Commun 342:293-9. 2006
    ..Humanized knock-in mice with codon 129 Val/Val were not susceptible. The results suggest that human heterozygotes at codon 129 are also at risk for secondary infection with vCJD...
  62. ncbi request reprint Beyond PrP res type 1/type 2 dichotomy in Creutzfeldt-Jakob disease
    Emmanuelle Uro-Coste
    INSERM U858, Institut de Médecine Moléculaire de Rangueil and Service d Anatomie Pathologique et Histologie Cytologie, C H U Rangueil, Toulouse, France
    PLoS Pathog 4:e1000029. 2008
    ....
  63. pmc Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob disease
    Emmanuelle Uro-Coste
    INSERM U858, Institut de Médecine Moléculaire de Rangueil and Service d Anatomie Pathologique et Histologie Cytologie, C H U Rangueil, Toulouse, France
    PLoS Pathog 4:e1000029. 2008
    ....
  64. pmc The first case of variant Creutzfeldt-Jakob disease in The Netherlands
    Paswal Sánchez-Juan
    J Neurol 254:958-60. 2007
  65. ncbi request reprint Redox metals and oxidative abnormalities in human prion diseases
    Robert B Petersen
    Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Acta Neuropathol 110:232-8. 2005
    ..These findings suggest an important distinction in prion-related oxidative stress, indicating that different neurodegenerative pathways are involved in different prion diseases...
  66. ncbi request reprint Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt-Jakob disease
    Atsushi Kobayashi
    Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, 2 1 Seiryo machi, Aoba ku, Sendai 980 8575, Japan
    J Gen Virol 86:237-40. 2005
    ..These results indicate that type 2 PrP(Sc) has a larger aggregation size than type 1, irrespective of the existence of amyloid plaques...
  67. ncbi request reprint Expression of excitatory amino acid transporter-1 (EAAT-1) in brain macrophages and microglia of patients with prion diseases
    Fabrice Chretien
    INSERM EMI0011 and Département de Pathologie, Hopital Henri Mondor, Assistance Publique Hôpital de Paris, Creteil, France
    J Neuropathol Exp Neurol 63:1058-71. 2004
    ..Whether this neuroprotective function of microglia is the cause or the effect of longer survival needs to be clarified...
  68. ncbi request reprint Prevalence of lymphoreticular prion protein accumulation in UK tissue samples
    David A Hilton
    Department of Histopathology, Derriford Hospital, Plymouth, UK
    J Pathol 203:733-9. 2004
    ....
  69. ncbi request reprint An outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases)
    Paweł P Liberski
    Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Poland
    Folia Neuropathol 42:39-58. 2004
    ..We also review the peculiarities of kuru, variant Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease...
  70. ncbi request reprint Complement activation in human prion disease
    Gabor G Kovacs
    Institute of Neurology, University of Vienna, Vienna, Austria
    Neurobiol Dis 15:21-8. 2004
    ..The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases...
  71. ncbi request reprint Mutations of the prion protein gene phenotypic spectrum
    Gabor G Kovacs
    Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria
    J Neurol 249:1567-82. 2002
    ..The codon 129 polymorphism has a phenotypic influence in inherited prion diseases, as in non-genetic forms, but additional factors might be considered as background for phenotypic variability...
  72. ncbi request reprint The brain smear, a rapid affordable intraoperative diagnostic technique for brain tumours appropriate for Africa
    Babatunde J Olasode
    Department of Pathology, Obafemi Awolowo University, PMB 5538, Ile Ife, Osun State
    Trop Doct 34:223-5. 2004
    ..It is hoped that the method can be introduced in areas where intraoperative diagnosis is required for brain tumours and the frozen section is not available...
  73. ncbi request reprint Transplantation of ocular tissue from a donor with sporadic Creutzfeldt-Jakob disease
    Andrew B Tullo
    Manchester Royal Eye Hospital, Manchester, UK
    Clin Experiment Ophthalmol 34:645-9. 2006
    ..We report an incident in the UK in 1997 in which both corneas and scleras from a donor, subsequently confirmed to have had sCJD, were transplanted. The final clinical outcome for two surviving recipients is still not yet known...