Search Results - "Wadsworth, Jonathan D.F."

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein by Asante, Emmanuel A., Linehan, Jacqueline M., Desbruslais, Melanie, Joiner, Susan, Gowland, Ian, Wood, Andrew L., Welch, Julie, Hill, Andrew F., Lloyd, Sarah E., Wadsworth, Jonathan D.F., Collinge, John

“…Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that…”
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Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein by Wadsworth, Jonathan D F, Joiner, Susan, Linehan, Jacqueline M, Balkema-Buschmann, Anne, Spiropoulos, John, Simmons, Marion M, Griffiths, Peter C, Groschup, Martin H, Hope, James, Brandner, Sebastian, Asante, Emmanuel A, Collinge, John

“…Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in…”
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Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease by Brandner, Sebastian, Whitfield, Jerome, Boone, Ken, Puwa, Anderson, O'Malley, Catherine, Linehan, Jacqueline M, Joiner, Susan, Scaravilli, Francesco, Calder, Ian, P. Alpers, Michael, Wadsworth, Jonathan D.F, Collinge, John

“…While the neuropathology of kuru is well defined, there are few data concerning the distribution of disease-related prion protein in peripheral tissues. Here…”
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Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy by Jaunmuktane, Zane, Mead, Simon, Ellis, Matthew, Wadsworth, Jonathan D. F., Nicoll, Andrew J., Kenny, Joanna, Launchbury, Francesca, Linehan, Jacqueline, Richard-Loendt, Angela, Walker, A. Sarah, Rudge, Peter, Collinge, John, Brandner, Sebastian

“…Treatment of children with human cadaver-derived growth hormone (c-hGH) contaminated with prions resulted in transmission of Creutzfeldt–Jakob disease (CJD);…”
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The origin of the prion agent of kuru: molecular and biological strain typing by Wadsworth, Jonathan D.F, Joiner, Susan, Linehan, Jacqueline M, Asante, Emmanuel A, Brandner, Sebastian, Collinge, John

“…Kuru is an acquired human prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. The central clinical…”
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Strain-specific prion-protein conformation determined by metal ions by Collinge, John, Wadsworth, Jonathan D.F, Hill, Andrew F, Joiner, Susan, Jackson, Graham S, Clarke, Anthony R

“…In animals infected with a transmissible spongiform encephalopathy, or prion disease, conformational isomers (known as PrPSc proteins) of the wild-type,…”
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Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129 by Mok, Tzehow, Jaunmuktane, Zane, Joiner, Susan, Campbell, Tracy, Morgan, Catherine, Wakerley, Benjamin, Golestani, Farhad, Rudge, Peter, Mead, Simon, Jäger, H. Rolf, Wadsworth, Jonathan D.F, Brandner, Sebastian, Collinge, John

“…In this case study, variant Creutzfeldt–Jakob disease (CJD) is shown to occur in a young man with heterozygosity, rather than homozygosity, at codon 129 of the…”
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A Novel Prion Disease Associated with Diarrhea and Autonomic Neuropathy by Mead, Simon, Gandhi, Sonia, Beck, Jon, Caine, Diana, Gallujipali, Dillip, Carswell, Christopher, Hyare, Harpreet, Joiner, Susan, Ayling, Hilary, Lashley, Tammaryn, Linehan, Jacqueline M, Al-Doujaily, Huda, Sharps, Bernadette, Revesz, Tamas, Sandberg, Malin K, Reilly, Mary M, Koltzenburg, Martin, Forbes, Alastair, Rudge, Peter, Brandner, Sebastian, Warren, Jason D, Wadsworth, Jonathan D.F, Wood, Nicholas W, Holton, Janice L, Collinge, John

“…Prions cause a variety of CNS illnesses, such as Creutzfeldt–Jakob disease. In this British kindred, a prion-associated process was associated with chronic…”
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Methods for Molecular Diagnosis of Human Prion Disease by Wadsworth, Jonathan D F, Adamson, Gary, Joiner, Susan, Brock, Lara, Powell, Caroline, Linehan, Jacqueline M, Beck, Jonathan A, Brandner, Sebastian, Mead, Simon, Collinge, John

“…Human prion diseases are associated with a range of clinical presentations, and they are classified by both clinicopathological syndrome and etiology, with…”
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Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein by Asante, Emmanuel A, Linehan, Jacqueline M, Smidak, Michelle, Tomlinson, Andrew, Grimshaw, Andrew, Jeelani, Asif, Jakubcova, Tatiana, Hamdan, Shyma, Powell, Caroline, Brandner, Sebastian, Wadsworth, Jonathan D F, Collinge, John

“…Prions are infectious agents causing fatal neurodegenerative diseases of humans and animals. In humans, these have sporadic, acquired and inherited…”
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Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein by Joiner, Susan, Asante, Emmanuel A., Linehan, Jacqueline M., Brock, Lara, Brandner, Sebastian, Bellworthy, Susan J., Simmons, Marion M., Hope, James, Collinge, John, Wadsworth, Jonathan D.F.

“…The epizootic prion disease of cattle, bovine spongiform encephalopathy (BSE), causes variant Creutzfeldt-Jakob disease (vCJD) in humans following dietary…”
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Human Prion Protein with Valine 129 Prevents Expression of Variant CJD Phenotype by Jonathan D. F. Wadsworth, Asante, Emmanuel A., Desbruslais, Melanie, Linehan, Jacqueline M., Joiner, Susan, Gowland, Ian, Welch, Julie, Stone, Lisa, Lloyd, Sarah E., Hill, Andrew F., Brandner, Sebastian, Collinge, John

“…Variant Creutzfeldt-Jakob disease (vCJD) is a unique and highly distinctive clinicopathological and molecular phenotype of human prion disease associated with…”
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Disease-related Prion Protein Forms Aggresomes in Neuronal Cells Leading to Caspase Activation and Apoptosis by Kristiansen, Mark, Messenger, Marcus J., Klöhn, Peter-Christian, Brandner, Sebastian, Wadsworth, Jonathan D.F., Collinge, John, Tabrizi, Sarah J.

“…The molecular basis for neuronal death in prion disease is not established, but putative pathogenic roles for both disease-related prion protein (PrPSc) and…”
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First Report of Creutzfeldt-Jakob Disease Occurring in 2 Siblings Unexplained by PRNP Mutation by Webb, Thomas E.F, Pal, Suvankar, Siddique, Durrenajaf, Heaney, Dominic C, Linehan, Jacqueline M, Wadsworth, Jonathan D.F, Joiner, Susan, Beck, Jon, Wroe, Stephen J, Stevenson, Valerie, Brandner, Sebastian, Mead, Simon, Collinge, John

“…Sibling concurrence of pathologically confirmed prion disease has only been reported in association with pathogenic mutation of the prion protein gene (PRNP)…”
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2.7 Å cryo-EM structure of ex vivo RML prion fibrils by Manka, Szymon W., Zhang, Wenjuan, Wenborn, Adam, Betts, Jemma, Joiner, Susan, Saibil, Helen R., Collinge, John, Wadsworth, Jonathan D. F.

“…Mammalian prions propagate as distinct strains and are composed of multichain assemblies of misfolded host-encoded prion protein (PrP). Here, we present a…”
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Prion strains viewed through the lens of cryo-EM by Manka, Szymon W., Wenborn, Adam, Collinge, John, Wadsworth, Jonathan D. F.

“…Mammalian prions are lethal transmissible pathogens that cause fatal neurodegenerative diseases in humans and animals. They consist of fibrils of misfolded,…”
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A structural basis for prion strain diversity by Manka, Szymon W., Wenborn, Adam, Betts, Jemma, Joiner, Susan, Saibil, Helen R., Collinge, John, Wadsworth, Jonathan D. F.

“…Recent cryogenic electron microscopy (cryo-EM) studies of infectious, ex vivo, prion fibrils from hamster 263K and mouse RML prion strains revealed a similar,…”
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Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years by Rudge, Peter, Jaunmuktane, Zane, Adlard, Peter, Bjurstrom, Nina, Caine, Diana, Lowe, Jessica, Norsworthy, Penny, Hummerich, Holger, Druyeh, Ron, Wadsworth, Jonathan D F, Brandner, Sebastian, Hyare, Harpreet, Mead, Simon, Collinge, John

“…Patients with iatrogenic Creutzfeldt-Jakob disease due to administration of cadaver-sourced growth hormone during childhood are still being seen in the UK 30…”
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Humanized Transgenic Mice Are Resistant to Chronic Wasting Disease Prions From Norwegian Reindeer and Moose by Wadsworth, Jonathan D F, Joiner, Susan, Linehan, Jacqueline M, Jack, Kezia, Al-Doujaily, Huda, Costa, Helena, Ingold, Thea, Taema, Maged, Zhang, Fuquan, Sandberg, Malin K, Brandner, Sebastian, Tran, Linh, Vikøren, Turid, Våge, Jørn, Madslien, Knut, Ytrehus, Bjørnar, Benestad, Sylvie L, Asante, Emmanuel A, Collinge, John

“…Abstract Chronic wasting disease (CWD) is the transmissible spongiform encephalopathy or prion disease affecting cervids. In 2016, the first cases of CWD were…”
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A naturally occurring variant of the human prion protein completely prevents prion disease by Asante, Emmanuel A., Smidak, Michelle, Grimshaw, Andrew, Houghton, Richard, Tomlinson, Andrew, Jeelani, Asif, Jakubcova, Tatiana, Hamdan, Shyma, Richard-Londt, Angela, Linehan, Jacqueline M., Brandner, Sebastian, Alpers, Michael, Whitfield, Jerome, Mead, Simon, Wadsworth, Jonathan D. F., Collinge, John

“…This study looks at a polymorphism of the human prion protein gene, which results in a G-to-V substitution at residue 127, in transgenic mice expressing…”
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