Search Results - "Haigh, Cathryn L."

Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction by Foliaki, Simote T, Smith, Anna, Schwarz, Benjamin, Bohrnsen, Eric, Bosio, Catharine M, Williams, Katie, Orrú, Christina D, Lachenauer, Hailey, Groveman, Bradley R, Haigh, Cathryn L

“…Fatal familial insomnia (FFI) is a rare neurodegenerative disease caused by a dominantly inherited single amino acid substitution (D178N) within the prion…”
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Prion propagation and cellular dysfunction in prion disease: Disconnecting the dots by Foliaki, Simote T, Haigh, Cathryn L

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Sodium hypochlorite inactivation of human CJD prions by Groveman, Bradley R, Race, Brent, Hughson, Andrew G, Haigh, Cathryn L

“…Prion diseases are transmissible, fatal neurologic diseases of mammals caused by the accumulation of mis-folded, disease associated prion protein (PrPd)…”
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Human cerebral organoids as a therapeutic drug screening model for Creutzfeldt–Jakob disease by Groveman, Bradley R., Ferreira, Natalia C., Foliaki, Simote T., Walters, Ryan O., Winkler, Clayton W., Race, Brent, Hughson, Andrew G., Zanusso, Gianluigi, Haigh, Cathryn L.

“…Creutzfeldt–Jakob Disease (CJD) is a fatal, currently incurable, neurodegenerative disease. The search for candidate treatments would be greatly facilitated by…”
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Lack of Transmission of Chronic Wasting Disease Prions to Human Cerebral Organoids by Groveman, Bradley R, Williams, Katie, Race, Brent, Foliaki, Simote, Thomas, Tina, Hughson, Andrew G, Walters, Ryan O, Zou, Wenquan, Haigh, Cathryn L

“…Chronic wasting disease (CWD) is a cervid prion disease with unknown zoonotic potential that might pose a risk to humans who are exposed. To assess the…”
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Cerebral organoids as a new model for prion disease by Groveman, Bradley R, Smith, Anna, Williams, Katie, Haigh, Cathryn L

“…Human brain has proven difficult to model, and this tissue is not widely available for study. [...]the application of organoid technology to produce 3D…”
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Stress and viral insults do not trigger E200K PrP conversion in human cerebral organoids by Smith, Anna, Groveman, Bradley R, Winkler, Clayton, Williams, Katie, Walters, Ryan, Yuan, Jue, Zou, Wenquan, Peterson, Karin, Foliaki, Simote T, Haigh, Cathryn L

“…Prion diseases are a group of rare, transmissible, and invariably fatal neurodegenerative diseases that affect both humans and animals. The cause of these…”
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Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids by Groveman, Bradley R, Foliaki, Simote T, Orru, Christina D, Zanusso, Gianluigi, Carroll, James A, Race, Brent, Haigh, Cathryn L

“…For the transmissible, neurogenerative family of prion diseases, few human models of infection exist and none represent structured neuronal tissue. Human…”
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α-Synuclein seeding activity in duodenum biopsies from Parkinson's disease patients by Vascellari, Sarah, Orrù, Christina D, Groveman, Bradley R, Parveen, Sabiha, Fenu, Giuseppe, Pisano, Giada, Piga, Giuseppe, Serra, Giulia, Oppo, Valentina, Murgia, Daniela, Perra, Andrea, Angius, Fabrizio, Hughson, Andrew G, Haigh, Cathryn L, Manzin, Aldo, Cossu, Giovanni, Caughey, Byron

“…Abnormal deposition of α-synuclein is a key feature and biomarker of Parkinson's disease. α-Synuclein aggregates can propagate themselves by a prion-like…”
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Hereditary E200K mutation within the prion protein gene alters human iPSC derived cardiomyocyte function by Wood, Aleksandar R., Foliaki, Simote T., Groveman, Bradley R., Walters, Ryan O., Williams, Katie, Yuan, Jue, Zou, Wen-Quan, Haigh, Cathryn L.

“…Cardiomyopathy is a co-morbidity of some prion diseases including genetic disease caused by mutations within the PrP gene ( PRNP ). Although the cellular prion…”
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Reduced SOD2 expression does not influence prion disease course or pathology in mice by Foliaki, Simote T, Race, Brent, Williams, Katie, Baune, Chase, Groveman, Bradley R, Haigh, Cathryn L

“…Prion diseases are progressive, neurodegenerative diseases affecting humans and animals. Also known as the transmissible spongiform encephalopathies, for the…”
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Neuronal maturation reduces the type I IFN response to orthobunyavirus infection and leads to increased apoptosis of human neurons by Winkler, Clayton W, Woods, Tyson A, Groveman, Bradley R, Carmody, Aaron B, Speranza, Emily E, Martens, Craig A, Best, Sonja M, Haigh, Cathryn L, Peterson, Karin E

“…La Crosse virus (LACV) is the leading cause of pediatric arboviral encephalitis in the USA. LACV encephalitis can result in learning and memory deficits, which…”
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Neuronal excitatory-to-inhibitory balance is altered in cerebral organoid models of genetic neurological diseases by Foliaki, Simote T, Schwarz, Benjamin, Groveman, Bradley R, Walters, Ryan O, Ferreira, Natalia C, Orrù, Christina D, Smith, Anna, Wood, Aleksandar, Schmit, Olivia M, Freitag, Phoebe, Yuan, Jue, Zou, Wenquan, Bosio, Catharine M, Carroll, James A, Haigh, Cathryn L

“…The neuro-physiological properties of individuals with genetic pre-disposition to neurological disorders are largely unknown. Here we aimed to explore these…”
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The Prion Protein N1 and N2 Cleavage Fragments Bind to Phosphatidylserine and Phosphatidic Acid; Relevance to Stress-Protection Responses by Haigh, Cathryn L, Tumpach, Carolin, Drew, Simon C, Collins, Steven J

“…Internal cleavage of the cellular prion protein generates two well characterised N-terminal fragments, N1 and N2. These fragments have been shown to bind to…”
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Neural cell engraftment therapy for sporadic Creutzfeldt-Jakob disease restores neuroelectrophysiological parameters in a cerebral organoid model by Williams, Katie, Foliaki, Simote T, Race, Brent, Smith, Anna, Thomas, Tina, Groveman, Bradley R, Haigh, Cathryn L

“…Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is a fatal neurodegenerative disease with currently no treatment options. Stem…”
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Using our mini-brains: cerebral organoids as an improved cellular model for human prion disease by Groveman, Bradley, Walters, Ryan, Haigh, Cathryn

“…The most common form of human prion disease is sporadic Creutzfeldt-Jakob disease (CJD), occurring in 1–2 people per million per year. Progression of prion…”
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Temporary alteration of neuronal network communication is a protective response to redox imbalance that requires GPI-anchored prion protein by Foliaki, Simote T., Wood, Aleksandar, Williams, Katie, Smith, Anna, Walters, Ryan O., Baune, Chase, Groveman, Bradley R., Haigh, Cathryn L.

“…Cellular prion protein (PrPC) protects neurons against oxidative stress damage. This role is lost upon its misfolding into insoluble prions in prion diseases,…”
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Sporadic Creutzfeldt-Jakob disease infected human cerebral organoids retain the original human brain subtype features following transmission to humanized transgenic mice by Groveman, Bradley R, Race, Brent, Foliaki, Simote T, Williams, Katie, Hughson, Andrew G, Baune, Chase, Zanusso, Gianluigi, Haigh, Cathryn L

“…Human cerebral organoids (COs) are three-dimensional self-organizing cultures of cerebral brain tissue differentiated from induced pluripotent stem cells. We…”
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Neutron Reflectometry Studies Define Prion Protein N-terminal Peptide Membrane Binding by Le Brun, Anton P., Haigh, Cathryn L., Drew, Simon C., James, Michael, Boland, Martin P., Collins, Steven J.

“…The prion protein (PrP), widely recognized to misfold into the causative agent of the transmissible spongiform encephalopathies, has previously been shown to…”
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Pathogenic Prion Protein Isoforms Are Not Present in Cerebral Organoids Generated from Asymptomatic Donors Carrying the E200K Mutation Associated with Familial Prion Disease by Foliaki, Simote, Groveman, Bradley, Yuan, Jue, Walters, Ryan, Zhang, Shulin, Tesar, Paul, Zou, Wenquan, Haigh, Cathryn

“…Cerebral organoids (COs) are a self-organizing three-dimensional brain tissue mimicking the human cerebral cortex. COs are a promising new system for modelling…”
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