James W Ironside

Summary

Affiliation: University of Edinburgh
Country: UK

Publications

  1. doi request reprint Variant Creutzfeldt-Jakob disease
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Haemophilia 16:175-80. 2010
  2. 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
  3. ncbi request reprint Phenotypic variability in human prion diseases
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, University of Edinburgh, UK
    Neuropathol Appl Neurobiol 31:565-79. 2005
  4. ncbi request reprint Variant Creutzfeldt-Jakob disease: risk of transmission by blood and blood products
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Edinburgh, UK
    Haemophilia 10:64-9. 2004
  5. ncbi request reprint Infective dementias
    J W Ironside
    Neuropathology Laboratory, Division of Pathology, School of Molecular and and Clinical Medicine, University of Edinburgh, Edimburgh, UK
    Panminerva Med 49:209-25. 2007
  6. ncbi request reprint Variant Creutzfeldt-Jakob disease: risk of transmission by blood transfusion and blood therapies
    J W Ironside
    University of Edinburgh, National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
    Haemophilia 12:8-15; discussion 26-8. 2006
  7. 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
  8. 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
  9. 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
  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

Publications104 found, 100 shown here

  1. doi request reprint Variant Creutzfeldt-Jakob disease
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Haemophilia 16:175-80. 2010
    ..Continued surveillance is required to investigate the prevalence of secondary variant CJD infection in other patients with bleeding disorders who have been treated with UK-sourced pooled plasma products...
  2. 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...
  3. ncbi request reprint Phenotypic variability in human prion diseases
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, University of Edinburgh, UK
    Neuropathol Appl Neurobiol 31:565-79. 2005
    ..Further work is required to clarify these relationships and to explain the mechanism of neuropathological targeting of specific brain regions, which accounts for the diversity of clinical features within human prion diseases...
  4. ncbi request reprint Variant Creutzfeldt-Jakob disease: risk of transmission by blood and blood products
    J W Ironside
    National Creutzfeldt Jakob Disease Surveillance Unit, Edinburgh, UK
    Haemophilia 10:64-9. 2004
    ....
  5. ncbi request reprint Infective dementias
    J W Ironside
    Neuropathology Laboratory, Division of Pathology, School of Molecular and and Clinical Medicine, University of Edinburgh, Edimburgh, UK
    Panminerva Med 49:209-25. 2007
    ..This review summarises the current state of knowledge regarding the major infective dementias...
  6. ncbi request reprint Variant Creutzfeldt-Jakob disease: risk of transmission by blood transfusion and blood therapies
    J W Ironside
    University of Edinburgh, National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
    Haemophilia 12:8-15; discussion 26-8. 2006
    ..In the absence of screening tests or effective therapies to treat this disease, a formidable worldwide public health challenge lies ahead to prevent new infections, accurately assess infection rates and treat infected patients...
  7. 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...
  8. 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...
  9. 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...
  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 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...
  12. 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...
  13. 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...
  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. 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
  16. 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...
  17. 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
    ....
  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 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...
  20. 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...
  21. ncbi request reprint Predicting susceptibility and incubation time of human-to-human transmission of vCJD
    M T Bishop
    National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh, UK
    Lancet Neurol 5:393-8. 2006
    ..We aimed to model iatrogenic spread to enable a comparison of transmission efficiencies of vCJD and bovine spongiform encephalopathy (BSE) and an assessment of the effect of the codon-129 polymorphism on human susceptibility...
  22. 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...
  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 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
    ....
  25. 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
    ....
  26. 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...
  27. 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
    ....
  28. doi request reprint Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia
    A Peden
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Edinburgh, UK
    Haemophilia 16:296-304. 2010
    ....
  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. 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
    ....
  32. 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...
  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. 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...
  35. 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...
  36. 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...
  37. 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...
  38. 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...
  39. 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...
  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. 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...
  42. 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
    ....
  43. ncbi request reprint In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc
    M Jones
    National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    J Pathol 213:21-6. 2007
    ....
  44. pmc Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease
    M W Head
    National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, University of Edinburgh EH4 2XU, UK
    Br J Ophthalmol 89:1131-3. 2005
    ..Involvement of the eye has been reported in patients with variant Creutzfeldt-Jakob disease (vCJD), but there is disagreement on whether retinal involvement occurs in sporadic Creutzfeldt-Jakob disease (sCJD)...
  45. 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
    ....
  46. ncbi request reprint Third ventricular chordoid glioma: clinicopathological study of two cases with evidence for a poor clinical outcome despite low grade histological features
    K M Kurian
    Neuropathology, Department of Pathology, Western General Hospital, Edinburgh, UK
    Neuropathol Appl Neurobiol 31:354-61. 2005
    ..Limited follow-up after surgery with or without radiotherapy suggests that as-full-as-possible resection favours a better outcome, although surgery in this area carries significant operative risks...
  47. ncbi request reprint Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent
    M E Bruce
    Institute for Animal Health, BBSRC MRC Neuropathogenesis Unit, Edinburgh, UK
    Nature 389:498-501. 1997
    ..Here we report the interim results of transmissions of sporadic CJD and vCJD to mice. Our data provide strong evidence that the same agent strain is involved in both BSE and vCJD...
  48. pmc Use of 14-3-3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease
    A J Green
    The National Creutzfeldt Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK
    J Neurol Neurosurg Psychiatry 70:744-8. 2001
    ..This study reports and compares the findings of CSF brain specific protein analysis in 45 patients with vCJD and in 34 control patients...
  49. 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...
  50. ncbi request reprint Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype
    M W Head
    Department of Pathology, University of Edinburgh, Western General Hospital, UK
    Ann Neurol 50:258-61. 2001
    ..Certain clinical and molecular features of this case overlap those of variant CJD. The case highlights possible difficulties in the differential diagnosis of vCJD and the more rare sCJD subtypes based on molecular features alone...
  51. ncbi request reprint Variant Creutzfeldt-Jakob disease
    J W Ironside
    National CJD Surveillance Unit, Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    Vet Res Commun 27:11-3. 2003
  52. ncbi request reprint Variant Creutzfeldt-Jakob disease: immunocytochemical studies and image analysis
    W H Nailon
    CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom
    Microsc Res Tech 50:2-9. 2000
    ..This technique has major potential as a research tool in human prion diseases, particularly for the characterisation of disease phenotype in large series of cases...
  53. ncbi request reprint Prion diseases in man
    J W Ironside
    Department of Pathology, University of Edinburgh, U K
    J Pathol 186:227-34. 1998
    ..Phenotypic variation is prominent in all forms of human prion disease; future classifications of these disorders are likely to incorporate genetic and biochemical data in addition to clinical and pathological parameters...
  54. 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
    ....
  55. 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...
  56. ncbi request reprint In situ hybridization analysis of PrP mRNA in human CNS tissues
    N F McLennan
    National CJD Surveillance Unit, Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Neuropathol Appl Neurobiol 27:373-83. 2001
    ..It is hoped that the information produced here will be helpful in understanding the pathology associated with CJD and other prion diseases in humans...
  57. ncbi request reprint Advances in the detection of prion protein in peripheral tissues of variant Creutzfeldt-Jakob disease patients using paraffin-embedded tissue blotting
    D L Ritchie
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Clinical and Molecular Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK
    Neuropathol Appl Neurobiol 30:360-8. 2004
    ..Autopsy and biopsy brain material from sporadic CJD cases also showed an increased sensitivity of PrP(res) detection with the PET blot, confirming its value as an important diagnostic and research tool in human prion diseases...
  58. 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
    ....
  59. ncbi request reprint Neuropathological findings in new variant CJD and experimental transmission of BSE
    J W Ironside
    CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
    FEMS Immunol Med Microbiol 21:91-5. 1998
    ....
  60. 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
    ....
  61. 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...
  62. 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...
  63. 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...
  64. pmc vCJD and the gut: implications for endoscopy
    M W Head
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    Gut 56:9-11. 2007
  65. 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
    ....
  66. ncbi request reprint Variant Creutzfeldt-Jakob disease
    H J T Ward
    National Creutzfeldt Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
    Clin Lab Med 23:87-108. 2003
    ..Indeed a recent publication has suggested that the drugs quinacrine or chloropromazine may be candidates for the treatment of human prion diseases [42]...
  67. ncbi request reprint Sporadic Creutzfeldt-Jakob disease in two adolescents
    K Murray
    National Creutzfeldt Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2 XU, UK
    J Neurol Neurosurg Psychiatry 79:14-8. 2008
    ..Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional...
  68. pmc Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK
    C A Heath
    National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh EH4 2XU, UK
    J Neurol Neurosurg Psychiatry 77:880-2. 2006
    ..The mean incubation period of the human dura mater cases was 93 (range 45-177) months. The clinico-pathological features of the cases are described and compared with cases previously reported in the world literature...
  69. pmc Raised CSF phospho-tau concentrations in variant Creutzfeldt-Jakob disease: diagnostic and pathological implications
    C A Goodall
    The National Creutzfeldt Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh, UK
    J Neurol Neurosurg Psychiatry 77:89-91. 2006
    ..To investigate whether phosphorylated tau protein (tau-pT181) is increased in variant Creutzfeldt-Jakob disease (vCJD) and if the tau-pT181/tau protein ratio is useful for distinguishing between patients with and without CJD...
  70. ncbi request reprint Investigation of PrPres in dental tissues in variant CJD
    M W Head
    National Creutzfeldt Jakob Disease Surveillance Unit, Division of Pathology, School of Clinical and Molecular Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
    Br Dent J 195:339-43; discussion 331. 2003
    ..To study the distribution of disease-associated prion protein (PrP) in oral and dental tissues in variant CJD...
  71. 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...
  72. 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
    ....
  73. ncbi request reprint Size frequency distributions of the florid prion protein aggregates in variant Creutzfeldt-Jakob disease follow a power-law function
    R A Armstrong
    Vision Sciences, Aston University, Birmingham B4 7ET, and National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
    Neurol Sci 27:104-9. 2006
    ..Processes of aggregation/disaggregation may be more important than surface diffusion in the pathogenesis of the florid plaques. By contrast, surface diffusion may be a more significant factor in the development of the diffuse plaques...
  74. ncbi request reprint Autoimmune hypothyroidism coexisting with a pituitary adenoma secreting thyroid-stimulating hormone, prolactin and alpha-subunit
    J M Idiculla
    Endocrine Department, Royal Infirmary, Edinburgh, UK
    Ann Clin Biochem 38:566-71. 2001
    ..This case illustrates the difficulty of diagnosing a TSHoma when it coexists with autoimmune hypothyroidism. We believe the combination of pathologies reported here is unique...
  75. ncbi request reprint Dynamics of reactive oxygen intermediate production in human glioma: n-6 essential fatty acid effects
    H A Leaver
    Department of Pharmacology, University of Edinburgh, UK
    Eur J Clin Invest 29:220-31. 1999
    ..Local essential fatty acid (EFA) deficiencies in tumour cells may limit tumour ROI generation. This deficiency may be rectified by the addition of exogenous EFA...
  76. ncbi request reprint Tissue and organ donation for research in forensic pathology: the MRC Sudden Death Brain and Tissue Bank
    T Millar
    Neuropathology Unit, University of Edinburgh, Alexander Donald Building, Western General Hospital, Edinburgh, EH4 2XU, UK
    J Pathol 213:369-75. 2007
    ..The potential for acquisition of normal CNS and non-CNS tissues and of various hard-to-get CNS disorders suggests that efforts to access the forensic post mortem service for research material are eminently worthwhile...
  77. ncbi request reprint The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease
    M Zeidler
    National Creutzfeldt Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, UK
    Lancet 355:1412-8. 2000
    ..We investigated the sensitivity and specificity of bilateral pulvinar high signal on magnetic resonance imaging (MRI) for the diagnosis of vCJD...
  78. ncbi request reprint An intramedullary vascular malformation mimicking intrinsic spinal cord tumour
    E W K Peng
    Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK
    Br J Neurosurg 20:109-10. 2006
  79. 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...
  80. 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...
  81. 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...
  82. 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...
  83. 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
    ....
  84. 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...
  85. 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
    ....
  86. 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...
  87. 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...
  88. 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...
  89. 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...
  90. 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
    ....
  91. 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...
  92. 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...
  93. 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
    ....
  94. 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
    ....
  95. 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...
  96. 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
  97. 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...
  98. pmc The first case of variant Creutzfeldt-Jakob disease in The Netherlands
    Paswal Sánchez-Juan
    J Neurol 254:958-60. 2007
  99. 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...
  100. 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...
  101. 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...