James W Ironside

James W Ironside

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James W Ironside

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A nonsynonymous mutation in PLCG2 reduces the risk of Alzheimer's disease, dementia with Lewy bodies and frontotemporal dementia, and increases the likelihood of longevity.

Authors:
Sven J van der Lee Olivia J Conway Iris Jansen Minerva M Carrasquillo Luca Kleineidam Erik van den Akker Isabel Hernández Kristel R van Eijk Najada Stringa Jason A Chen Anna Zettergren Till F M Andlauer Monica Diez-Fairen Javier Simon-Sanchez Alberto Lleó Henrik Zetterberg Marianne Nygaard Cornelis Blauwendraat Jeanne E Savage Jonas Mengel-From Sonia Moreno-Grau Michael Wagner Juan Fortea Michael J Keogh Kaj Blennow Ingmar Skoog Manuel A Friese Olga Pletnikova Miren Zulaica Carmen Lage Itziar de Rojas Steffi Riedel-Heller Ignacio Illán-Gala Wei Wei Bernard Jeune Adelina Orellana Florian Then Bergh Xue Wang Marc Hulsman Nina Beker Niccolo Tesi Christopher M Morris Begoña Indakoetxea Lyduine E Collij Martin Scherer Estrella Morenas-Rodríguez James W Ironside Bart N M van Berckel Daniel Alcolea Heinz Wiendl Samantha L Strickland Pau Pastor Eloy Rodríguez Rodríguez Bradley F Boeve Ronald C Petersen Tanis J Ferman Jay A van Gerpen Marcel J T Reinders Ryan J Uitti Lluís Tárraga Wolfgang Maier Oriol Dols-Icardo Amit Kawalia Maria Carolina Dalmasso Mercè Boada Uwe K Zettl Natasja M van Schoor Marian Beekman Mariet Allen Eliezer Masliah Adolfo López de Munain Alexander Pantelyat Zbigniew K Wszolek Owen A Ross Dennis W Dickson Neill R Graff-Radford David Knopman Rosa Rademakers Afina W Lemstra Yolande A L Pijnenburg Philip Scheltens Thomas Gasser Patrick F Chinnery Bernhard Hemmer Martijn A Huisman Juan Troncoso Fermin Moreno Ellen A Nohr Thorkild I A Sørensen Peter Heutink Pascual Sánchez-Juan Danielle Posthuma Jordi Clarimón Kaare Christensen Nilüfer Ertekin-Taner Sonja W Scholz Alfredo Ramirez Agustín Ruiz Eline Slagboom Wiesje M van der Flier Henne Holstege

Acta Neuropathol 2019 Aug 27;138(2):237-250. Epub 2019 May 27.

Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1007/s00401-019-02026-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6660501PMC
August 2019

Variably protease-sensitive prionopathy mimicking frontotemporal dementia.

Neuropathology 2019 Apr 7;39(2):135-140. Epub 2019 Mar 7.

Clinical Neuropathology, King's College Hospital, NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1111/neup.12538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6778052PMC
April 2019

Neuropathology of Human Prion Diseases.

Prog Mol Biol Transl Sci 2017 3;150:319-339. Epub 2017 Aug 3.

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/bs.pmbts.2017.06.011DOI Listing
May 2018

Prion diseases.

Handb Clin Neurol 2017 ;145:393-403

National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom.

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http://dx.doi.org/10.1016/B978-0-12-802395-2.00028-6DOI Listing
January 2018

Human stem cell-derived astrocytes replicate human prions in a genotype-dependent manner.

J Exp Med 2017 Dec 15;214(12):3481-3495. Epub 2017 Nov 15.

Medical Research Council Centre for Regenerative Medicine, University of Edinburgh, Edinburgh, Scotland, UK

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http://dx.doi.org/10.1084/jem.20161547DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5716027PMC
December 2017

-glycosylation and expression in human tissues of the orphan GPR61 receptor.

FEBS Open Bio 2017 12 20;7(12):1982-1993. Epub 2017 Nov 20.

Institute of Clinical Sciences College of Medical and Dental Sciences University of Birmingham UK.

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http://dx.doi.org/10.1002/2211-5463.12339DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5715243PMC
December 2017

Infectivity in bone marrow from sporadic CJD patients.

J Pathol 2017 11 22;243(3):273-278. Epub 2017 Sep 22.

UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, France.

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http://dx.doi.org/10.1002/path.4954DOI Listing
November 2017

Gerstmann-Sträussler-Scheinker disease with atypical presentation.

BMJ Case Rep 2017 Nov 1;2017. Epub 2017 Nov 1.

Department of Neurology, Ninewells Hospital, Dundee, Tayside, UK.

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2017-22090
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http://dx.doi.org/10.1136/bcr-2017-220907DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5695393PMC
November 2017

Amyloid-β accumulation in the CNS in human growth hormone recipients in the UK.

Acta Neuropathol 2017 08 27;134(2):221-240. Epub 2017 Mar 27.

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine, University of Edinburgh, Edinburgh, EH4 2XU, UK.

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http://dx.doi.org/10.1007/s00401-017-1703-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5508038PMC
August 2017

Multisite Assessment of Aging-Related Tau Astrogliopathy (ARTAG).

J Neuropathol Exp Neurol 2017 Jul;76(7):605-619

Institute of Neurology, Medical University of Vienna, Vienna, Austria; Center for Neurodegenerative Disease Research, Institute on Aging and Department of Pathology and Laboratory Medicine of the Perelman School of Medicine at the University of Pennsylvania; and Department of Biostatistics and Epidemiology; and Department of Neurosurgery, Center for Brain Injury and Repair, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden; Institute of Neuroscience, Newcastle University, Newcastle upon Tyne, UK; Department of Neuropathology, Institute of Pathology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida; Northwestern University Feinberg School of Medicine, Northwestern ADC Neuropathology Core, Chicago, Illinois; Clinical Neuropathology, King's College Hospital and London Neurodegenerative Brain Bank, London, UK; Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland; University of California San Francisco, Institute for Neurodegenerative Diseases, San Francisco, California; Neuropathology Department, Hôpital de La Salpetrière, AP-HP, UPMC-Sorbonne-University, Paris, France; Institute of Neuropathology, Bellvitge University Hospital, University of Barcelona, CIBERNED, Hospitalet de Llobregat, Barcelona, Spain; Discipline of Pathology, Sydney Medical School, The University of Sydney, Sydney, Australia; Neurological Tissue Bank of the Biobank-Hospital Clinic-IDIBAPS, Institut d'Investigacions Biomediques Pi i, Barcelona, Spain; Department of Medicine, Imperial College London, London, UK; IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy; Memory and Aging Center, Department of Neurology, University of California, San Francisco, California; Department of Pathology, University of Sao Paulo Medical School, LIM, São Paulo, Brazil; Brain & Mind Centre, Sydney Medical School, The University of Sydney, and UNSW Medicine & NeuRA, Sydney, Australia; Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas; Fishberg Department of Neuroscience, Friedman Brain Institute, and Ronald M. Loeb Center for Alzheimer's Disease, Icahn School of Medicine at Mount Sinai, New York, New York; Department of Neuropathology, John Radcliffe Hospital, Oxford, UK; Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK; Department of Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania; Department of Mental Health and Psychiatry, University Hospitals and University of Geneva School of Medicine, Geneva, Switzerland; Institute of Clinical Neurosciences, University of Bristol, Learning & Research Level 2, Southmead Hospital, Bristol, UK; Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada; Department of Pathology and Molecular Medicine, Thomayer Hospital, Prague, Czech Republic; Department of Pathology, First Medical Faculty, Charles University, Prague, Czech Republic; Department of Anatomical Pathology, Alfred Hospital , Prahran, Victoria, Australia; Division of Pathology, St. Michael's Hospital, Toronto, Ontario, Canada; Department of Pathology and Sanders-Brown Center on Aging, University of Kentucky, Lexington, Kentucky; Physiopathology in Aging Lab/Brazilian Aging Brain Study Group-LIM22, University of Sao Paulo Medical School, Sao Paulo, Brazil; Behavioral and Cognitive Neurology Unit, Department of Neurology, University of São Paulo , São Paulo, Brazil; Netherlands Brainbank, Amsterdam and Department of Pathology, VU University Medical Center, Amsterdam, The Netherlands; Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan; Institute of Neuroanatomy, Centre for Biomedicine and Medical Technology Mannheim, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany; Department of Neurodegenerative Diseases and Gerontopsychiatry at the University of Bonn Medical Center, Bonn, Germany; Department of Pathology, Brain Research Institute, Niigata University, Niigata, Japan; Department of Neurology, Saitama Medical University International Medical Center, Saitama, Japan; Department of Neuroscience, Katholieke Universiteit-Leuven; and Department of Pathology, Universitaire Ziekenhuizen-Leuven, Leuven, Belgium; Laboratory of Neuropathology, Department of Pathology and Neuropathology, Kepler University Hospital, Medical School, Johannes Kepler University, Linz, Austria; Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK; and Department of Pathology and Laboratory Medicine, Centre for Cancer Therapeutics, Ottawa Hospital Research Institute, University of Ottawa, Ontario, Canada.

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http://dx.doi.org/10.1093/jnen/nlx041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251511PMC
July 2017

UK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.

Acta Neuropathol 2017 04 3;133(4):579-595. Epub 2016 Nov 3.

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Sciences, The University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1007/s00401-016-1638-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5348565PMC
April 2017

Response to Simon et al.

Acta Neuropathol Commun 2017 04 29;5(1):34. Epub 2017 Apr 29.

Department of Clinical Neurosciences and MRC Mitochondrial Biology Unit, University of Cambridge, Cambridge, UK.

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http://dx.doi.org/10.1186/s40478-017-0434-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5410018PMC
April 2017

Reply: Atherosclerosis and vascular cognitive impairment neuropathological guideline.

Brain 2017 02 21;140(2):e13. Epub 2016 Dec 21.

Dementia Research Group, School of Clinical Sciences, Faculty of Health Sciences, University of Bristol, Level 1, Learning & Research, Southmead Hospital, Bristol, BS10 5NB, UK

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http://dx.doi.org/10.1093/brain/aww305DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5841058PMC
February 2017

Sporadic and Infectious Human Prion Diseases.

Cold Spring Harb Perspect Med 2017 Jan 3;7(1). Epub 2017 Jan 3.

National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom.

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http://perspectivesinmedicine.cshlp.org/lookup/doi/10.1101/c
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http://dx.doi.org/10.1101/cshperspect.a024364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5204333PMC
January 2017

Relative Abundance of apoE and Aβ1-42 Associated with Abnormal Prion Protein Differs between Creutzfeldt-Jakob Disease Subtypes.

J Proteome Res 2016 12 30;15(12):4518-4531. Epub 2016 Sep 30.

Rocky Mountain Laboratories, National Institute of Allergy & Infectious Disease, National Institutes of Health , Hamilton, Montana 59840, United States.

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http://dx.doi.org/10.1021/acs.jproteome.6b00633DOI Listing
December 2016

Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease.

Sci Transl Med 2016 12;8(370):370ra183

Mitchell Center for Alzheimer's Disease and Related Brain Disorders, University of Texas Houston Medical School, Houston, TX 77030, USA.

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http://dx.doi.org/10.1126/scitranslmed.aaf6188DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5538786PMC
December 2016

Vascular cognitive impairment neuropathology guidelines (VCING): the contribution of cerebrovascular pathology to cognitive impairment.

Brain 2016 11;139(11):2957-2969

Dementia Research Group, School of Clinical Sciences, Faculty of Health Sciences, University of Bristol, Learning and Research Level 1, Southmead Hospital, Bristol, BS10 5NB, UK.

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http://dx.doi.org/10.1093/brain/aww214DOI Listing
November 2016

Alpha-synuclein RT-QuIC in the CSF of patients with alpha-synucleinopathies.

Ann Clin Transl Neurol 2016 10 28;3(10):812-818. Epub 2016 Aug 28.

The National CJD Research & Surveillance Unit Western General Hospital University of Edinburgh Edinburgh EH4 2XU United Kingdom.

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http://dx.doi.org/10.1002/acn3.338DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5048391PMC
October 2016

Sacrificing the superior petrosal vein during microvascular decompression. Is it safe? Learning the hard way. Case report and review of literature.

Surg Neurol Int 2016 3;7(Suppl 14):S415-20. Epub 2016 Jun 3.

Department of Neurosurgery, Aberdeen Royal Infirmary, NHS Grampian, Edinburgh, Scotland, UK.

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http://dx.doi.org/10.4103/2152-7806.183520DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4901823PMC
June 2016

A Naturally Occurring Bovine Tauopathy Is Geographically Widespread in the UK.

PLoS One 2015 19;10(6):e0129499. Epub 2015 Jun 19.

Animal and Plant Health Agency, Lasswade Veterinary Laboratory, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, Scotland.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0129499PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4474601PMC
April 2016

Blood transmission studies of prion infectivity in the squirrel monkey (Saimiri sciureus): the Baxter study.

Transfusion 2016 Mar 23;56(3):712-21. Epub 2015 Nov 23.

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland.

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http://dx.doi.org/10.1111/trf.13422DOI Listing
March 2016

The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients.

PLoS Pathog 2016 Feb 3;12(2):e1005416. Epub 2016 Feb 3.

Rocky Mountain Laboratories, National Institute of Allergy & Infectious Diseases, National Institutes of Health, Hamilton, Montana, United States of America.

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http://dx.doi.org/10.1371/journal.ppat.1005416DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4740439PMC
February 2016

Current concepts in the prevention of pathogen transmission via blood/plasma-derived products for bleeding disorders.

Blood Rev 2016 Jan 20;30(1):35-48. Epub 2015 Jul 20.

National Creutzfeldt-Jakob Disease Research and Surveillance Unit, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK.

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https://linkinghub.elsevier.com/retrieve/pii/S0268960X150005
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http://dx.doi.org/10.1016/j.blre.2015.07.004DOI Listing
January 2016

Aging-related tau astrogliopathy (ARTAG): harmonized evaluation strategy.

Acta Neuropathol 2016 Jan 10;131(1):87-102. Epub 2015 Dec 10.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.

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http://dx.doi.org/10.1007/s00401-015-1509-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4879001PMC
January 2016

Variant CJD. 18 years of research and surveillance.

Prion 2014 1;8(4):286-95. Epub 2014 Nov 1.

a The Roslin Institute and R(D)SVS ; University of Edinburgh; Easter Bush ; Midlothian , Scotland , UK.

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http://dx.doi.org/10.4161/pri.29237DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4601215PMC
October 2015

Pathological and biochemical investigation of a woman diagnosed with genetic Creutzfeldt-Jakob disease shortly after parturition.

Neuropathol Appl Neurobiol 2015 Aug 2;41(5):676-80. Epub 2015 May 2.

Institute of Neurology, Medical University of Vienna, Vienna, Austria.

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http://dx.doi.org/10.1111/nan.12204DOI Listing
August 2015

A case of variably protease-sensitive prionopathy treated with doxycyclin.

J Neurol Neurosurg Psychiatry 2015 Jul 9;86(7):816-8. Epub 2015 Jan 9.

Institute of Neurology, Medical University of Vienna and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria.

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http://dx.doi.org/10.1136/jnnp-2014-309871DOI Listing
July 2015

BrainNet Europe's Code of Conduct for brain banking.

J Neural Transm (Vienna) 2015 Jul 13;122(7):937-40. Epub 2015 Jan 13.

Netherlands Brain Bank, Netherlands Institute for Neuroscience, Meibergdreef 47, 1105 BA, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1007/s00702-014-1353-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4498226PMC
July 2015

Uptake and degradation of protease-sensitive and -resistant forms of abnormal human prion protein aggregates by human astrocytes.

Am J Pathol 2014 Dec 30;184(12):3299-307. Epub 2014 Sep 30.

Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, Montana. Electronic address:

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http://dx.doi.org/10.1016/j.ajpath.2014.08.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4258502PMC
December 2014

The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease.

Acta Neuropathol Commun 2014 Oct 21;2:152. Epub 2014 Oct 21.

National CJD Research & Surveillance Unit (NCJDRSU), School of Clinical Sciences (Division of Clinical Neurosciences), College of Medicine and Veterinary Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK.

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http://dx.doi.org/10.1186/s40478-014-0152-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4210614PMC
October 2014

Human prion diseases and the risk of their transmission during anatomical dissection.

Clin Anat 2014 Sep 17;27(6):821-32. Epub 2014 Apr 17.

The Roslin Institute and R(D)SVS The University of Edinburgh, Midlothian EH25 9RG, United Kingdom.

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http://dx.doi.org/10.1002/ca.22403DOI Listing
September 2014

Investigation of variant Creutzfeldt-Jakob disease implicated organ or tissue transplantation in the United Kingdom.

Transplantation 2014 Sep;98(5):585-9

1 National CJD Research & Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK. 2 Scottish National Blood Transfusion Service Tissues and Cells Directorate, Aberdeen, UK. 3 NHS Blood and Transplant, London, UK. 4 Scottish National Blood Transfusion Service Tissues and Cells Directorate, Edinburgh, UK. 5 NHS National Services Scotland Public Health and Intelligence, Edinburgh, UK. 6 Address correspondence to: Anna Molesworth, M.Sc., Ph.D., National CJD Research & Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK.

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http://pdfs.journals.lww.com/transplantjournal/2014/09150/In
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http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
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http://dx.doi.org/10.1097/TP.0000000000000105DOI Listing
September 2014

Genotype-dependent molecular evolution of sheep bovine spongiform encephalopathy (BSE) prions in vitro affects their zoonotic potential.

J Biol Chem 2014 Sep 6;289(38):26075-88. Epub 2014 Aug 6.

From the National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh, Edinburgh EH4 2XU, United Kingdom,

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http://dx.doi.org/10.1074/jbc.M114.582965DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4176218PMC
September 2014

Comparative Study of Prions in Iatrogenic and Sporadic Creutzfeldt-Jakob Disease.

J Clin Cell Immunol 2014 Aug;5(4)

Department of Pathology and National Prion Disease, Pathology Surveillance Center, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; Department of Neurology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; National Center for Regenerative Medicine, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; The First Affiliated Hospital, Nanchang University, Nanchang, Jiangxi Province, The People's Republic of China.

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http://dx.doi.org/10.4172/2155-9899.1000240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4240320PMC
August 2014

Sporadic Creutzfeldt-Jakob disease presenting as progressive nonfluent aphasia with speech apraxia.

Alzheimer Dis Assoc Disord 2013 Oct-Dec;27(4):384-6

*Cerebral Function Unit §Department of Neuroradiology, Greater Manchester Neurosciences Centre, Salford †Mental Health and Neurodegeneration Research Group, School of Community-Based Medicine ‡School of Cancer and Enabling Sciences, University of Manchester, Manchester ∥Department of Neurology, Royal Preston Hospital, Preston ¶National CJD Research and Surveillance Unit, Western General Hospital, Edinburgh, UK.

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http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
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http://dx.doi.org/10.1097/WAD.0b013e318260ab27DOI Listing
July 2014

Gerstmann-Straüssler-Scheinker disease: novel PRNP mutation and VGKC-complex antibodies.

Neurology 2014 Jun 9;82(23):2107-11. Epub 2014 May 9.

From the Greater Manchester Neurosciences Centre (M.J., D.d.P., D.G.), Salford Royal Foundation Trust, Salford; Manchester Medical School (S.O., D.d.P.), and Institute of Brain, Behaviour and Mental Health (M.J.), University of Manchester; Nuffield Department of Clinical Neurosciences (A.V.), John Radcliffe Hospital, University of Oxford; and National Creutzfeldt-Jakob Disease Research and Surveillance Unit (M.B., M.W.H., J.W.I.), University of Edinburgh, UK.

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http://dx.doi.org/10.1212/WNL.0000000000000500DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4118501PMC
June 2014

Human tonsil-derived follicular dendritic-like cells are refractory to human prion infection in vitro and traffic disease-associated prion protein to lysosomes.

Am J Pathol 2014 Jan 1;184(1):64-70. Epub 2013 Nov 1.

National Creutzfeldt-Jakob Disease Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.ajpath.2013.09.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3873479PMC
January 2014

Brain banking for neurological disorders.

Lancet Neurol 2013 Nov 25;12(11):1096-105. Epub 2013 Sep 25.

Division of Clinical Neurosciences, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1016/S1474-4422(13)70202-3DOI Listing
November 2013

Pathogen safety of long-term treatments for bleeding disorders: (un)predictable risks and evolving threats.

Semin Thromb Hemost 2013 Oct 12;39(7):779-93. Epub 2013 Sep 12.

Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Napoli, Italy.

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http://dx.doi.org/10.1055/s-0033-1354420DOI Listing
October 2013

Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes.

J Biol Chem 2013 Jul 21;288(30):21659-66. Epub 2013 Jun 21.

Department of Neurological Science, Tohoku University Graduate School of Medicine, 2-1, Seiryo-machi, Aoba-ku, Sendai 980-8575, Japan.

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http://dx.doi.org/10.1074/jbc.M113.470328DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3724625PMC
July 2013

Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease.

Brain 2013 Apr 28;136(Pt 4):1139-45. Epub 2013 Feb 28.

National Creutzfeldt–Jakob Disease Research and Surveillance Unit, University of Edinburgh, Bryan Matthews Building, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK.

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https://academic.oup.com/brain/article-lookup/doi/10.1093/br
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http://dx.doi.org/10.1093/brain/awt032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3613713PMC
April 2013

Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008.

Brain 2013 Apr;136(Pt 4):1102-15

National Creutzfeldt-Jakob Disease Research & Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh EH4 2XU, UK.

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http://dx.doi.org/10.1093/brain/aws366DOI Listing
April 2013

Endocrine mucin-producing sweat gland carcinoma: report of two cases of an under-recognized malignant neoplasm and review of the literature.

Am J Dermatopathol 2013 Feb;35(1):117-24

Department of Pathology, Western General Hospital, Edinburgh, United Kingdom.

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http://dx.doi.org/10.1097/DAD.0b013e31826367beDOI Listing
February 2013

Molecular pathology in neurodegenerative diseases.

Curr Drug Targets 2012 Nov;13(12):1548-59

National CJD Research & Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK.

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http://dx.doi.org/10.2174/138945012803530134DOI Listing
November 2012

Variant Creutzfeldt-Jakob disease: an update.

Authors:
James W Ironside

Folia Neuropathol 2012 ;50(1):50-6

National Creutzfeldt-Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, EH4 2XU, United Kingdom.

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August 2012

The contribution of different prion protein types and host polymorphisms to clinicopathological variations in Creutzfeldt-Jakob disease.

Rev Med Virol 2012 Jul 28;22(4):214-29. Epub 2012 Feb 28.

The National CJD Research & Surveillance Unit, School of Molecular & Clinical Medicine, University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1002/rmv.725DOI Listing
July 2012

Bilateral non-contiguous atypical papillary glioneuronal tumor: case report.

Clin Neuropathol 2012 Mar-Apr;31(2):77-80

Department of Neurosurgery, University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.5414/np300391DOI Listing
July 2012

Creutzfeldt-Jakob disease.

Adv Exp Med Biol 2012 ;724:76-90

Department of Molecular Pathology and Neuropathology, Medical University Lodz, Lodz, Poland.

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http://dx.doi.org/10.1007/978-1-4614-0653-2_6DOI Listing
April 2012

The molecular epidemiology of variant CJD.

Int J Mol Epidemiol Genet 2011 Aug 3;2(3):217-27. Epub 2011 Jun 3.

National CJD Research and Surveillance unit, Western General Hospital Crewe road, Edinburgh, EH4 2XU, UK.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3166149PMC
August 2011

Heparin enhances the cell-protein misfolding cyclic amplification efficiency of variant Creutzfeldt-Jakob disease.

Neurosci Lett 2011 Jul 5;498(2):119-23. Epub 2011 May 5.

Research and Development Division, Benesis Corporation, c/o Division of Neurological Science, Department of Prion Research, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Japan.

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http://dx.doi.org/10.1016/j.neulet.2011.04.072DOI Listing
July 2011

Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species.

Brain Pathol 2011 May 3;21(3):298-307. Epub 2010 Nov 3.

National Creutzfeldt-Jakob Disease Surveillance Unit, School of Molecular and Clinical Medicine (Pathology), University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1111/j.1750-3639.2010.00446.xDOI Listing
May 2011

Human embryonic stem cells rapidly take up and then clear exogenous human and animal prions in vitro.

J Pathol 2011 Apr 21;223(5):635-45. Epub 2011 Feb 21.

National CJD Surveillance Unit, School of Molecular and Clinical Medicine (Pathology), University of Edinburgh, Western General Hospital, Edinburgh, UK.

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http://dx.doi.org/10.1002/path.2832DOI Listing
April 2011

31st Advanced Clinical Neurology Course, Edinburgh 2009: progressive cognitive impairment, behavioural change and upper motor neuron signs in a 57-year-old woman.

Pract Neurol 2011 Apr;11(2):71-80

Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK.

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http://dx.doi.org/10.1136/jnnp.2011.241786DOI Listing
April 2011

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.

J Gen Virol 2011 Mar 1;92(Pt 3):727-32. Epub 2010 Dec 1.

National CJD Surveillance Unit, School of Molecular & Clinical Medicine (Pathology), University of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1099/vir.0.026948-0DOI Listing
March 2011

Effect of fixation on brain and lymphoreticular vCJD prions and bioassay of key positive specimens from a retrospective vCJD prevalence study.

J Pathol 2011 Mar 10;223(4):511-8. Epub 2010 Dec 10.

MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.

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http://dx.doi.org/10.1002/path.2821DOI Listing
March 2011

The application of in vitro cell-free conversion systems to human prion diseases.

Acta Neuropathol 2011 Jan 10;121(1):135-43. Epub 2010 Jun 10.

Components and vCJD Research, National Science Laboratories, Scottish National Blood Transfusion Service, Edinburgh, EH 17 7QT, UK.

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http://dx.doi.org/10.1007/s00401-010-0708-8DOI Listing
January 2011

A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702).

Neuro Oncol 2010 Dec 22;12(12):1257-68. Epub 2010 Sep 22.

Children's Brain Tumour Research Centre, Division of Human Development, Queen's Medical Centre, University of Nottingham, Nottingham.

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http://dx.doi.org/10.1093/neuonc/noq092DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3018938PMC
December 2010

Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay.

J Virol 2010 Nov 15;84(22):12030-8. Epub 2010 Sep 15.

National CJD Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK.

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http://dx.doi.org/10.1128/JVI.01057-10DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2977900PMC
November 2010

Two cases of recurrent stroke in treated giant cell arteritis: diagnostic and therapeutic dilemmas.

J Clin Rheumatol 2010 Aug;16(5):225-8

Department of Rehabilitation Medicine, Astley Ainslie Hospital, Edinburgh, Scotland.

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https://insights.ovid.com/crossref?an=00124743-201008000-000
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http://dx.doi.org/10.1097/RHU.0b013e3181e9a338DOI Listing
August 2010

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.

J Gen Virol 2009 Dec 5;90(Pt 12):3075-82. Epub 2009 Aug 5.

National CJD Surveillance Unit, School of Molecular and Clinical Medicine (Pathology), University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK.

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http://dx.doi.org/10.1099/vir.0.013227-0DOI Listing
December 2009

Molecular model of prion transmission to humans.

Emerg Infect Dis 2009 Dec;15(12):2013-6

National CJD Surveillance Unit, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, Scotland EH4 2XU, UK.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3044515PMC
http://dx.doi.org/10.3201/eid1512.090194DOI Listing
December 2009

Differential protein profiling as a potential multi-marker approach for TSE diagnosis.

BMC Infect Dis 2009 Nov 27;9:188. Epub 2009 Nov 27.

The Roslin Institute & R(D)SVS, University of Edinburgh, Roslin, Midlothian, EH25 9PS, UK.

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http://dx.doi.org/10.1186/1471-2334-9-188DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2794872PMC
November 2009

Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease?

Brain 2009 Oct 16;132(Pt 10):2627-9. Epub 2009 Sep 16.

National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh.

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http://dx.doi.org/10.1093/brain/awp225DOI Listing
October 2009