TACKLING KEY EPIDEMIOLOGICAL AND BASIC SCIENTIFIC RESEARCH ISSUES IN PRION DISEASES.

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

2023-03-11

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

2022-03-15

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

2022-01-01

INTRODUCTION: Fatty acid-binding protein 3 (FABP3) is a biomarker of neuronal membrane disruption, associated with lipid dyshomeos..

Prion disease in Indigenous Australians

Peter K Panegyres, Christiane Stehmann, Genevieve M Klug, Colin L Masters, Steven Collins

2021-07-01

BACKGROUND: Indigenous Australians are at increased risk of developing dementia - Alzheimer disease and mixed dementia diagnoses a..

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

2021-03-01

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

2020-11-01

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

2020-10-01

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

2020-07-07

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

2020-03-31

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

2020-02-01

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

2020-01-01

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

2020-01-01

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

2019-04-01

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

2019-03-21

Anthracyclines are amongst the most effective chemotherapeutics ever developed, but they produce grueling side-effects, serious ad..

Ethical Issues in the Treatment of Late-Stage Alzheimer's Disease

Andrew D Watt, Nicole L Jenkins, Gawain McColl, Steven Collins, Patricia M Desmond

2019-01-01

There is hope that the continuing efforts of researchers will yield a disease-modifying drug for Alzheimer's disease. Such a drug ..

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

2019-01-01

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

2018-09-01

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

2018-08-01

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

2018-04-01

The pre-mortem clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is supported by biomarkers, especially cerebrospinal fluid (C..

Acute Neurotoxicity Models of Prion Disease

AMT Islam, PA Adlard, DI Finkelstein, V Lewis, S Biggi, E Biasini, SJ Collins

2018-03-01

Prion diseases are phenotypically diverse, transmissible, neurodegenerative disorders affecting both animals and humans. Misfoldin..

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

2018-01-08

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

2017-07-01

The primary causative event in the development of prion diseases is the misfolding of the normal prion protein (PrPC) into an ense..