Research Topics
Genomes and Genes | James W IronsideSummaryAffiliation: University of Edinburgh Country: UK Publications
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Publications
BSE and vCJD cause disturbance to uric acid levelsTamuna 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...- Review: Creutzfeldt-Jakob diseaseJ W Ironside
Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
Brain Pathol 6:379-88. 1996.... - Variant Creutzfeldt-Jakob disease and its transmission by bloodJ 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.... 
Best Practice No 172: pituitary gland pathologyJ 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....- Neuropathology and molecular biology of variant Creutzfeldt-Jakob diseaseJ 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... - Pathological diagnosis of variant Creutzfeldt-Jakob diseaseJ W Ironside
Department of Pathology, University of Edinburgh, Western General Hospital, United Kingdom
APMIS 110:79-87. 2002.... - Neuropathology of variant Creutzfeldt-Jakob diseaseJames 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.... - Neuropathology of variant Creutzfeldt-Jakob diseaseJames 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... - Laboratory diagnosis of variant Creutzfeldt-Jakob diseaseJ 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... 
Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assayMichael Jones
National CJD Surveillance Unit, School of Molecular and Clinical Medicine Pathology, The Roslin Institute, Edinburgh, UK
Neuroreport 19:1783-6. 2008....- An antibody to the aggregated synthetic prion protein peptide (PrP106-126) selectively recognizes disease-associated prion protein (PrP) from human brain specimensMichael 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... - 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 miceDiane 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... - Molecular model of prion transmission to humansMichael 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... - Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric speciesYoung 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.... - Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002Mark 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... - Sporadic Creutzfeldt-Jakob disease: further twists and turns in a convoluted proteinMark W Head
National Creutzfeldt-Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2 XU, UK
Brain 129:2238-40. 2006 - Review: pathology of variant Creutzfeldt-Jakob diseaseAlexander 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... - Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence studyJames 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... - Abnormal prion protein in the pituitary in sporadic and variant Creutzfeldt-Jakob diseaseAlexander 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.... - Detection of type 1 prion protein in variant Creutzfeldt-Jakob diseaseHelen 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... - No major change in vCJD agent strain after secondary transmission via blood transfusionMatthew 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... - Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob diseaseMichael Jones
University of Edinburgh, Western General Hospital, UK
Transfusion 49:376-84. 2009.... - Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassayYoung 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... - Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative, and biochemical studyMark 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... - The application of in vitro cell-free conversion systems to human prion diseasesMichael Jones
Components and vCJD Research, National Science Laboratories, Scottish National Blood Transfusion Service, Edinburgh, EH 17 7QT, UK
Acta Neuropathol 121:135-43. 2011.... - Risks of transmission of variant Creutzfeldt-Jakob disease by blood transfusionAlexander 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.... - The spectrum of safety: variant Creutzfeldt-Jakob disease in the United KingdomJames 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... - Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversionAlexander 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... - Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob diseaseAlexander 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.... - Human embryonic stem cells rapidly take up and then clear exogenous human and animal prions in vitroZuzana 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.... - 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 - Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patientAlexander 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... - 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 blotYoung 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... - Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob diseaseMatthew 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... - Variably protease-sensitive prionopathy in the U.K.: a retrospective review 1991-2008Mark 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.... - 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 diseasePaula 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... - Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob diseaseMark 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..Given that routine decontamination may not remove PrP(Sc) from surgical instruments, a careful risk assessment should be made of possible iatrogenic spread of sporadic and variant CJD after surgery to the retina or optic nerve... - Further characterisation of the prion protein molecular types detectable in the NIBSC Creutzfeldt-Jakob disease brain reference materialsHelen 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... - Prion protein accumulation and neuroprotection in hypoxic brain damageNeil 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... - Management of a twenty-first century brain bank: experience in the BrainNet Europe consortiumJeanne E Bell
Department of Pathology Neuropathology, University of Edinburgh, Wilkie Building, Teviot Place, Edinburgh, UK
Acta Neuropathol 115:497-507. 2008.... - Application of Atomic Dielectric Resonance Spectroscopy for the screening of blood samples from patients with clinical variant and sporadic CJDTimothy 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... - Validation of diagnostic criteria for variant Creutzfeldt-Jakob diseaseCraig 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... - Differential protein profiling as a potential multi-marker approach for TSE diagnosisJanice 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... - The contribution of different prion protein types and host polymorphisms to clinicopathological variations in Creutzfeldt-Jakob diseaseMark 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.... - Variant Creutzfeldt-Jakob disease: an updateJames 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... - Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed casesDonald A Collie
Department of Neuroradiology, Western General Hospital, Edinburgh, Scotland
AJNR Am J Neuroradiol 24:1560-9. 2003..FLAIR sequence is more sensitive than other sequences. Positive MR images may obviate more invasive diagnostic tests in most cases... - Molecular pathology in neurodegenerative diseasesAlexander 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... - Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypesGabor 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... - Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseasesKatsuya 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... - Accumulation of prion protein in tonsil and appendix: review of tissue samplesDavid A Hilton
Department of Histopathology, Derriford Hospital, Plymouth PL6 8DH
BMJ 325:633-4. 2002 - Molecular classification of sporadic Creutzfeldt-Jakob diseaseAndrew 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... - Correlative studies support lipid peroxidation is linked to PrP(res) propagation as an early primary pathogenic event in prion diseaseMarcus W Brazier
Department of Pathology, The University of Melbourne, Vic. 3010, Australia
Brain Res Bull 68:346-54. 2006.... - 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... - Disease-associated prion protein in vessel wallsOskar 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... - Tetracyclines affect prion infectivityGianluigi Forloni
Istituto di Ricerche Farmacologiche Mario Negri, Istituto Nazionale Neurologico Carlo Besta, and Istituto di Microbiologia e Immunologia Veterinaria, , 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... - Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prionsTetsuyuki 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... - PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob DiseaseJames 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... - Increased clusterin (apolipoprotein J) expression in human and mouse brains infected with transmissible spongiform encephalopathiesKensuke Sasaki
Department of Neuropathology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
Acta Neuropathol 103:199-208. 2002.... - Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prionsYuzuru 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... - The first case of variant Creutzfeldt-Jakob disease in The NetherlandsPaswal Sánchez-Juan
J Neurol 254:958-60. 2007 - Redox metals and oxidative abnormalities in human prion diseasesRobert 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... - Beyond PrP res type 1/type 2 dichotomy in Creutzfeldt-Jakob diseaseEmmanuelle 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.... - vCJD prion acquires altered virulence through trans-species infectionMasahiro 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... - Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob diseaseEmmanuelle 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.... - Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt-Jakob diseaseAtsushi 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... - Expression of excitatory amino acid transporter-1 (EAAT-1) in brain macrophages and microglia of patients with prion diseasesFabrice 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... - Prevalence of lymphoreticular prion protein accumulation in UK tissue samplesDavid A Hilton
Department of Histopathology, Derriford Hospital, Plymouth, UK
J Pathol 203:733-9. 2004.... - Transplantation of ocular tissue from a donor with sporadic Creutzfeldt-Jakob diseaseAndrew B Tullo
Manchester Royal Eye Hospital, Manchester, UK
Clin Experiment Ophthalmol 34:645-9. 2006..Coupled with continuing concerns in the UK about person-to-person transmission of variant CJD, this has lead to a number of important consequences in donor eye retrieval, ocular tissue banking and transplantation... - Complement activation in human prion diseaseGabor 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... - Mutations of the prion protein gene phenotypic spectrumGabor 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... - The brain smear, a rapid affordable intraoperative diagnostic technique for brain tumours appropriate for AfricaBabatunde 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... - 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...