DISEASES OF THE AGEING BRAIN

Simplified Murine 3D Neuronal Cultures for Investigating Neuronal Activity and Neurodegeneration

Steven J Collins, Cathryn L Haigh

2017-03-01

The ability to model brain tissue in three-dimensions offers new potential for elucidating functional cellular interactions and co..

Pathogenic mechanisms of prion protein, amyloid-beta and alpha-synuclein misfolding: the prion concept and neurotoxicity of protein oligomers

Cathryn L Ugalde, David I Finkelstein, Victoria A Lawson, Andrew F Hill

2016-10-01

Proteinopathies represent a group of diseases characterized by the unregulated misfolding and aggregation of proteins. Accumulatio..

A 2-Substituted 8-Hydroxyquinoline Stimulates Neural Stem Cell Proliferation by Modulating ROS Signalling

Cathryn L Haigh, Carolin Tumpach, Steven J Collins, Simon C Drew

2016-09-01

Eight-hydroxyquinolines (8HQs) are a class of compounds that have been identified as potential therapeutics for a number of neurod..

Advances in the Development of Disease-Modifying Treatments for Amyotrophic Lateral Sclerosis

Diane Moujalled, Anthony R White

2016-03-01

Amyotrophic lateral sclerosis (ALS) is a progressive adult-onset, neurodegenerative disease characterized by the degeneration of u..

The prion protein regulates beta-amyloid-mediated self-renewal of neural stem cells in vitro

Steven J Collins, Carolin Tumpach, Qiao-Xin Li, Victoria Lewis, Timothy M Ryan, Blaine Roberts, Simon C Drew, Victoria A Lawson, Cathryn L Haigh

2015-04-11

The beta-amyloid (Aβ) peptide and the Aβ-oligomer receptor, prion protein (PrP), both influence neurogenesis. Using in vitro murin..

iSRAP - a one-touch research tool for rapid profiling of small RNA-seq data

Camelia Quek, Chol-hee Jung, Shayne A Bellingham, Andrew Lonie, Andrew F Hill

2015-01-01

Small non-coding RNAs have been significantly recognized as the key modulators in many biological processes, and are emerging as p..

In vivo evaluation of a novel tau imaging tracer for Alzheimer's disease

Victor L Villemagne, Shozo Furumoto, Michelle T Fodero-Tavoletti, Rachel S Mulligan, John Hodges, Ryuichi Harada, Paul Yates, Olivier Piguet, Svetlana Pejoska, Vincent Dore, Kazuhiko Yanai, Colin L Masters, Yukitsuka Kudo, Christopher C Rowe, Nobuyuki Okamura

2014-05-01

PURPOSE: Diagnosis of tauopathies such as Alzheimer's disease (AD) still relies on post-mortem examination of the human brain. A n..

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

Leslie I Grad, Justin J Yerbury, Bradley J Turner, William C Guest, Edward Pokrishevsky, Megan A O'Neill, Anat Yanai, Judith M Silverman, Rafaa Zeineddine, Lisa Corcoran, Janet R Kumita, Leila M Luheshi, Masoud Yousefi, Bradley M Coleman, Andrew F Hill, Steven S Plotkin, Ian R Mackenzie, Neil R Cashman

2014-03-04

Amyotrophic lateral sclerosis (ALS) is predominantly sporadic, but associated with heritable genetic mutations in 5-10% of cases, ..

Pathogenic Mutations within the Hydrophobic Domain of the Prion Protein Lead to the Formation of Protease-Sensitive Prion Species with Increased Lethality

Bradley M Coleman, Christopher F Harrison, Belinda Guo, Colin L Masters, Kevin J Barnham, Victoria A Lawson, Andrew F Hill

2014-03-01

UNLABELLED: Prion diseases are a group of fatal and incurable neurodegenerative diseases affecting both humans and animals. The pr..

Neuroprotective Copper Bis(thiosemicarbazonato) Complexes Promote Neurite Elongation

Laura Bica, Jeffrey R Liddell, Paul S Donnelly, Clare Duncan, Aphrodite Caragounis, Irene Volitakis, Brett M Paterson, Roberto Cappai, Alexandra Grubman, James Camakaris, Peter J Crouch, Anthony R White

2014-02-28

Abnormal biometal homeostasis is a central feature of many neurodegenerative disorders including Alzheimer's disease (AD), Parkins..

Deregulation of biometal homeostasis: the missing link for neuronal ceroid lipofuscinoses?

Alexandra Grubman, Eveliina Pollari, Clare Duncan, Aphrodite Caragounis, Tea Blom, Irene Volitakis, Andrew Wong, Jonathan Cooper, Peter J Crouch, Jari Koistinaho, Anu Jalanko, Anthony R White, Katja M Kanninen

2014-01-01

Neuronal ceroid lipofuscinoses (NCLs), a group of genetically distinct fatal neurodegenerative disorders with no treatment or cure..

X-ray fluorescence imaging reveals subcellular biometal disturbances in a childhood neurodegenerative disorder

A Grubman, SA James, J James, C Duncan, I Volitakis, JL Hickey, PJ Crouch, PS Donnelly, KM Kanninen, JR Liddell, SL Cotman, MD de Jonge, AR White

2014-01-01

Biometals such as zinc, iron, copper and calcium play key roles in diverse physiological processes in the brain, but can be toxic ..

Unusual Clinical and Molecular-Pathological Profile of Gerstmann-Straussler-Scheinker Disease Associated With a Novel PRNP Mutation (V176G)

Marion Simpson, Vanessa Johanssen, Alison Boyd, Genevieve Klug, Colin L Masters, Qiao-Xin Li, Roger Pamphlett, Catriona McLean, Victoria Lewis, Steven J Collins

2013-09-01

IMPORTANCE: Here we describe the unusual clinical and molecular-neuropathological profile of a case of Gerstmann-Sträussler-Schein..

Cytosolic caspases mediate mislocalised SOD2 depletion in an in vitro model of chronic prion infection

Layla Sinclair, Victoria Lewis, Steven J Collins, Cathryn L Haigh

2013-07-01

Oxidative stress as a contributor to neuronal death during prion infection is supported by the fact that various oxidative damage ..

Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis

Katja M Kanninen, Alexandra Grubman, Aphrodite Caragounis, Clare Duncan, Sarah J Parker, Grace E Lidgerwood, Irene Volitakis, George Ganio, Peter J Crouch, Anthony R White

2013-06-15

Neuronal ceroid lipofuscinoses, the most common fatal childhood neurodegenerative illnesses, share many features with more prevale..

Oligomers, fact or artefact? SDS-PAGE induces dimerization of beta-amyloid in human brain samples

Andrew D Watt, Keyla A Perez, Alan Rembach, Nicki A Sherrat, Lin Wai Hung, Timothy Johanssen, Catriona A McLean, Woan Mei Kok, Craig A Hutton, Michelle Fodero-Tavoletti, Colin L Masters, Victor L Villemagne, Kevin J Barnham

2013-04-01

The formation of low-order oligomers of β-amyloid (Aβ) within the brain is widely believed to be a central component of Alzheimer'..

Prion-infected cells regulate the release of exosomes with distinct ultrastructural features

Bradley M Coleman, Eric Hanssen, Victoria A Lawson, Andrew F Hill

2012-10-01

Exosomes are small membrane-bound vesicles released from cells and found in vivo in most biological fluids. Functions reported for..

POLYGLUTAMINE AGGREGATION IN HUNTINGTON AND RELATED DISEASES

Saskia Polling, Andrew F Hill, Danny M Hatters

2012-01-01

Polyglutamine (polyQ)-expansions in different proteins cause nine neurodegenerative diseases. While polyQ aggregation is a key pat..