TACKLING KEY EPIDEMIOLOGICAL AND BASIC SCIENTIFIC RESEARCH ISSUES IN PRION DISEASES.
Grant number: 1105784 | Funding period: 2016 - 2020
Proposed Practitioner Fellowship research will simultaneously include epidemiological and basic scientific projects, emphasising human prion disease. Epidemiological research will involve large-scale studies to clarify genetic factors that influence susceptibility to developing prion disease, as well as clarify whether Alzheimer disease is transmissible, while basic scientific research will include determining the characteristics of the misfolded prion proteins causing neuronal disease.
Related publications (22)
Tailored behavioural tests reveal early and progressive cognitive deficits in M1000 prion disease.
Matteo Senesi, Victoria Lewis, Paul A Adlard, David I Finkelstein, Jee Hyun Kim, Steven J Collins
Prion diseases are pathogenically linked to the normal cellular prion protein (PrPC) misfolding into abnormal conformers (PrPSc), ..
Diagnostic accuracy of cerebrospinal fluid biomarkers in genetic prion diseases
Matthias Schmitz, Anna Villar-Pique, Peter Hermann, Georgia Escaramis, Miguel Calero, Cao Chen, Niels Kruse, Maria Cramm, Ewa Golanska, Beata Sikorska, Pawel P Liberski, Maurizio Pocchiari, Peter Lange, Christiane Stehmann, Shannon Sarros, Eulalia Marti, Ines Baldeiras, Isabel Santana, Dana Zakova, Eva Mitrova
Genetic prion diseases are a rare and diverse group of fatal neurodegenerative disorders caused by pathogenic sequence variations ..
Cerebrospinal fluid levels of fatty acid-binding protein 3 are associated with likelihood of amyloidopathy in cognitively healthy individuals.
Kunal Dhiman, Victor L Villemagne, Christopher Fowler, Pierrick Bourgeat, Qiao-Xin Li, Steven Collins, Christopher C Rowe, Colin L Masters, David Ames, Kaj Blennow, Henrik Zetterberg, Ralph N Martins, Veer Gupta
INTRODUCTION: Fatty acid-binding protein 3 (FABP3) is a biomarker of neuronal membrane disruption, associated with lipid dyshomeos..
Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease
Peter Hermann, Brian Appleby, Jean-Philippe Brandel, Byron Caughey, Steven Collins, Michael D Geschwind, Alison Green, Stephane Haik, Gabor G Kovacs, Anna Ladogana, Franc Llorens, Simon Mead, Noriyuki Nishida, Suvankar Pal, Piero Parchi, Maurizio Pocchiari, Katsuya Satoh, Gianluigi Zanusso, Inga Zerr
Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrPSc). Effective ther..
Intra-cerebral haemorrhage but not neurodegenerative disease appears over-represented in deaths of Australian cadaveric pituitary hormone recipients
Sultan H Alnakhli, Handan Wand, Matthew Law, Shannon Sarros, Christiane Stehmann, Matteo Senesi, Genevieve M Klug, Marion Simpson, Victoria Lewis, Colin L Masters, Steven J Collins
BACKGROUND AND PURPOSE: Recent scientific reports and epidemiological studies have engendered mounting concerns regarding the pote..
Identification of novel risk loci and causal insights for sporadic Creutzfeldt-Jakob disease: a genome-wide association study
Emma Jones, Holger Hummerich, Emmanuelle Vir, James Uphill, Athanasios Dimitriadis, Helen Speedy, Tracy Campbell, Penny Norsworthy, Liam Quinn, Jerome Whitfield, Jacqueline Linehan, Zane Jaunmuktane, Sebastian Brandner, Parmjit Jat, Akin Nihat, Tze How Mok, Parvin Ahmed, Steven Collins, Christiane Stehmann, Shannon Sarros
Background Human prion diseases are rare and usually rapidly fatal neurodegenerative disorders, the most common being sporadic Cre..
PrPSc Oligomerization Appears Dynamic, Quickly Engendering Inherent M1000 Acute Synaptotoxicity
Simote T Foliaki, Victoria Lewis, Abu MT Islam, Matteo Senesi, David Finkelstein, Laura J Ellett, Victoria A Lawson, Paul A Adlard, Blaine R Roberts, Steven J Collins
Prion diseases are neurodegenerative disorders pathogenically linked to cellular prion protein (PrPC) misfolding into abnormal con..
Elecsys CSF biomarker immunoassays demonstrate concordance with amyloid-PET imaging
James D Doecke, Larry Ward, Samantha C Burnham, Victor L Villemagne, Qiao-Xin Li, Steven Collins, Christopher J Fowler, Ekaterina Manuilova, Monika Widmann, Stephanie R Rainey-Smith, Ralph N Martins, Colin L Masters
BACKGROUND: β-amyloid (Aβ) positron emission tomography (PET) imaging is currently the only Food and Drug Administration-approved ..
Diagnostic Accuracy of Prion Disease Biomarkers in Iatrogenic Creutzfeldt-Jakob Disease
Franc Llorens, Anna Villar-Pique, Peter Hermann, Matthias Schmitz, Olga Calero, Christiane Stehmann, Shannon Sarros, Fabio Moda, Isidre Ferrer, Anna Poleggi, Maurizio Pocchiari, Marcella Catania, Sigrid Klotz, Carl O'Regan, Francesca Brett, Josephine Heffernan, Anna Ladogana, Steven J Collins, Miguel Calero, Gabor G Kovacs
Human prion diseases are classified into sporadic, genetic, and acquired forms. Within this last group, iatrogenic Creutzfeldt-Jak..
Cerebrospinal fluid neurofilament light concentration predicts brain atrophy and cognition in Alzheimer's disease
Kunal Dhiman, Veer Bala Gupta, Victor L Villemagne, Dhamidhu Eratne, Petra L Graham, Christopher Fowler, Pierrick Bourgeat, Qiao-Xin Li, Steven Collins, Ashley Bush, Christopher C Rowe, Colin L Masters, David Ames, Eugene Hone, Kaj Blennow, Henrik Zetterberg, Ralph N Martins
INTRODUCTION: This study assessed the utility of cerebrospinal fluid (CSF) neurofilament light (NfL) in Alzheimer's disease (AD) d..
Markers of A1 astrocytes stratify to molecular sub-types in sporadic Creutzfeldt-Jakob disease brain
Cathryn L Ugalde, Victoria Lewis, Christiane Stehmann, Catriona A McLean, Victoria A Lawson, Steven J Collins, Andrew F Hill
Astrocytes are glial cells of the central nervous system that become reactive under conditions of stress. The functional propertie..
Early existence and biochemical evolution characterise acutely synaptotoxic PrPSc
Simote Totauhelotu Foliaki, Victoria Lewis, Abu Mohammed Taufiqual Islam, Laura Jane Ellett, Matteo Senesi, David Isaac Finkelstein, Blaine Roberts, Victoria A Lawson, Paul Anthony Adlard, Steven John Collins
Although considerable evidence supports that misfolded prion protein (PrPSc) is the principal component of "prions", underpinning ..
Secreted cellular prion protein binds doxorubicin and correlates with anthracycline resistance in breast cancer
Adrian P Wiegmans, Jodi M Saunus, Sunyoung Ham, Richard Lobb, Jamie R Kutasovic, Andrew J Dalley, Mariska Miranda, Caroline Atkinson, Simote T Foliaki, Kaltin Ferguson, Colleen Niland, Cameron N Johnstone, Victoria Lewis, Steven J Collins, Sunil R Lakhani, Fares Al-Ejeh, Andreas Moller
Anthracyclines are amongst the most effective chemotherapeutics ever developed, but they produce grueling side-effects, serious ad..
Supranutritional Sodium Selenate Supplementation Delivers Selenium to the Central Nervous System: Results from a Randomized Controlled Pilot Trial in Alzheimer's Disease
Barbara R Cardoso, Blaine R Roberts, Charles B Malpas, Lucy Vivash, Sila Genc, Michael M Saling, Patricia Desmond, Christopher Steward, Rodney J Hicks, Jason Callahan, Amy Brodtmann, Steven Collins, Stephen Macfarlane, Niall M Corcoran, Christopher M Hovens, Dennis Velakoulis, Terence J O'Brien, Dominic J Hare, Ashley I Bush
Insufficient supply of selenium to antioxidant enzymes in the brain may contribute to Alzheimer's disease (AD) pathophysiology; th..
Prion protein cleavage fragments regulate adult neural stem cell quiescence through redox modulation of mitochondrial fission and SOD2 expression
Steven J Collins, Carolin Tumpach, Bradley R Groveman, Simon C Drew, Cathryn L Haigh
Neurogenesis continues in the post-developmental brain throughout life. The ability to stimulate the production of new neurones re..
Prion acute synaptotoxicity is largely driven by protease-resistant PrPSc species
Simote Totauhelotu Foliaki, Victoria Lewis, David Isaac Finkelstein, Victoria Lawson, Harold Arthur Coleman, Matteo Senesi, Abu Mohammed Taufiqual Islam, Feng Chen, Shannon Sarros, Blaine Roberts, Paul Anthony Adlard, Steven John Collins
Although misfolding of normal prion protein (PrPC) into abnormal conformers (PrPSc) is critical for prion disease pathogenesis our..
CSF Tau supplements 14-3-3 protein detection for sporadic Creutzfeldt-Jakob disease diagnosis while transitioning to next generation diagnostics
Qiao-Xin Li, Shiji Varghese, Shannon Sarros, Christiane Stehmann, James D Doecke, Christopher J Fowler, Colin L Masters, Steven J Collins
The pre-mortem clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is supported by biomarkers, especially cerebrospinal fluid (C..
Iatrogenic Creutzfeldt-Jakob disease with Amyloid-beta pathology: an international study
Ignazio Cali, Mark L Cohen, Stephane Haik, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Veronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob d..
In vivo prion models and the disconnection between transmissibility and neurotoxicity
Matteo Senesi, Victoria Lewis, Jee H Kim, Paul A Adlard, David I Finkelstein, Steven J Collins
The primary causative event in the development of prion diseases is the misfolding of the normal prion protein (PrPC) into an ense..