Yerbury, Justin Professor

Member Of

• Proteostasis and Disease Research Centre Member 2012 -
• Illawarra Health and Medical Research Institute Member 2011 -
• Centre for Medical and Molecular Bioscience 2010 -

Has Collaborator

• Ecroyd, Heath  Available as Research Supervisor
• Ooi, Lezanne  Professor
• Perrow, Kara  Dr
• Ranson, Marie  Professor
• Sluyter, Ronald  Faculty Academic
• Wilson, Mark  Senior Professor

Selected Publications

• Journal Article

  Year	Title
  2022	A copper chaperone–mimetic polytherapy for SOD1-associated amyotrophic lateral sclerosis Published In Journal of Biological Chemistry
  2022	Cells Overexpressing ALS-Associated SOD1 Variants Are Differentially Susceptible to CuATSM-Associated Toxicity Published In ACS Chemical Neuroscience
  2022	P2X7 receptor activation mediates superoxide dismutase 1 (SOD1) release from murine NSC-34 motor neurons Published In Purinergic Signalling
  2022	Proteostasis impairment and ALS Published In Progress in Biophysics and Molecular Biology
  2022	TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex Published In Neurobiology of Disease
  2022	Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateral sclerosis? Published In Neural Regeneration Research
  2022	Vulnerability of the spinal motor neuron presynaptic terminal sub-proteome in ALS Published In Neuroscience Letters
  2021	Behavioural effects of cage systems on the G93A Superoxide Dismutase 1 transgenic mouse model for amyotrophic lateral sclerosis Published In Genes, Brain and Behavior
  2021	CuATSM improves motor function and extends survival but is not tolerated at a high dose in SOD1 G93A mice with a C57BL/6 background Published In Scientific Reports
  2021	Disabled in academia: to be or not to be, that is the question Published In Trends in Neurosciences
  2021	Mutant Cu/Zn Superoxide Dismutase (A4V) Turnover Is Altered in Cells Containing Inclusions Published In Frontiers in Molecular Neuroscience
  2021	Non-Viral Vector-Mediated Gene Therapy for ALS: Challenges and Future Perspectives Published In Molecular Pharmaceutics
  2021	Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations in CCNF Reveals Activation of the Apoptosis Pathway: A Workflow to Screen Pathogenic Gene Mutations Published In Frontiers in Molecular Neuroscience
  2021	p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death Published In Scientific Reports
  2020	Amyotrophic Lateral Sclerosis: Proteins, Proteostasis, Prions, and Promises Published In Frontiers in Cellular Neuroscience
  2020	Corrigendum: Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis (Frontiers in Molecular Neuroscience, (2019), 12, 10.3389/fnmol.2019.00262) Published In Frontiers in Molecular Neuroscience
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells Published In Journal of cell science
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells. Published In J Cell Sci
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal-like cells
  2020	Novel behavioural characteristics of the superoxide dismutase 1 G93A (SOD1G93A) mouse model of amyotrophic lateral sclerosis include sex-dependent phenotypes Published In Genes, Brain and Behavior
  2020	Proteome Homeostasis Dysfunction: A Unifying Principle in ALS Pathogenesis Published In Trends in Neurosciences
  2020	Selenium-based compounds: Emerging players in the ever-unfolding story of SOD1 in amyotrophic lateral sclerosis. Published In EBioMedicine
  2020	The P2X7 receptor antagonist JNJ-47965567 administered thrice weekly from disease onset does not alter progression of amyotrophic lateral sclerosis in SOD1G93A mice Published In Purinergic Signalling
  2020	The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins Published In Glia
  2020	The pivotal role of ubiquitin-activating enzyme E1 (UBA1) in neuronal health and neurodegeneration Published In International Journal of Biochemistry and Cell Biology
  2020	The prion-like nature of amyotrophic lateral sclerosis
  2020	Trajectory Taken by Dimeric Cu/Zn Superoxide Dismutase through the Protein Unfolding and Dissociation Landscape Is Modulated by Salt Bridge Formation Published In Analytical Chemistry
  2020	Tryptophan residue 32 in human Cu-Zn superoxide dismutase modulates prion-like propagation and strain selection Published In PLoS One
  2020	Ubiquitin Homeostasis Is Disrupted in TDP-43 and FUS Cell Models of ALS Published In iScience
  2019	Dynamic interplay between H-current and M-current controls motoneuron hyperexcitability in amyotrophic lateral sclerosis Published In Cell Death and Disease
  2019	Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis Published In Frontiers in Molecular Neuroscience
  2019	Strategies to promote the maturation of ALS-associated SOD1 mutants: Small molecules return to the fold Published In Neural Regeneration Research
  2019	The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation Published In Cells
  2019	The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS Published In Neuroscience Letters
  2019	Using Tetracysteine-Tagged TDP-43 with a Biarsenical Dye To Monitor Real-Time Trafficking in a Cell Model of Amyotrophic Lateral Sclerosis Published In Biochemistry
  2018	Assessment of metal concentrations in the SOD1G93Amouse model of amyotrophic lateral sclerosis and its potential role in muscular denervation, with particular focus on muscle tissue Published In Molecular and Cellular Neuroscience
  2018	Association of Regulatory T-Cell Expansion With Progression of Amyotrophic Lateral Sclerosis: A Study of Humans and a Transgenic Mouse Model Published In Jama Neurology
  2018	CuATSM Protects Against the in Vitro Cytotoxicity of Wild-Type-Like Copper-Zinc Superoxide Dismutase Mutants but not Mutants That Disrupt Metal Binding Published In ACS Chemical Neuroscience
  2018	Dysregulation of microRNA biogenesis machinery and microRNA/RNA ratio in skeletal muscle of amyotrophic lateral sclerosis mice Published In Muscle and Nerve
  2018	Nucleo-cytoplasmic transport of TDP-43 studied in real time: impaired microglia function leads to axonal spreading of TDP-43 in degenerating motor neurons Published In Acta Neuropathologica
  2018	Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48‑linked ubiquitylation and defective autophagy Published In Cellular and Molecular Life Sciences
  2018	SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS Published In Journal of Cell Science
  2018	Some diseases, like mine, deteriorate rapidly – disability services need to keep up Published In The Conversation
  2018	The cysteine-reactive small molecule ebselen facilitates effective SOD1 maturation Published In Nature Communications
  2018	Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells Published In Scientific Reports
  2017	Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation Published In Cell Stress and Chaperones
  2017	Casein kinase II phosphorylation of cyclin F at serine 621 regulates the Lys48-ubiquitylation E3 ligase activity of the SCF(cyclin F) complex Published In Open Biology
  2017	Clusterin protects neurons against intracellular proteotoxicity Published In Acta Neuropathologica Communications
  2017	Flow cytometric measurement of the cellular propagation of TDP-43 aggregation Published In Prion
  2017	Improving the Delivery of SOD1 Antisense Oligonucleotides to Motor Neurons Using Calcium Phosphate-Lipid Nanoparticles Published In Frontiers in Neuroscience
  2017	Longitudinal assessment of metal concentrations and copper isotope ratios in the G93A SOD1 mouse model of amyotrophic lateral sclerosis Published In Metallomics: integrated biometal science
  2017	Motor neuron disease proteins activate complement and generate C5a Published In MOLECULAR IMMUNOLOGY
  2017	P2X7 antagonism using Brilliant Blue G reduces body weight loss and prolongs survival in female SOD1G93A amyotrophic lateral sclerosis mice Published In PeerJ
  2017	P2X7 receptor antagonism in amyotrophic lateral sclerosis Published In Neural Regeneration Research
  2017	Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS Published In Proceedings of the National Academy of Sciences of USA
  2017	The heat shock response in neurons and astroglia and its role in neurodegenerative diseases Published In Molecular Neurodegeneration
  2017	The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis Published In BBA: Molecular Basis of Disease
  2016	CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia Published In Nature Communications
  2016	Disease mechanisms in ALS: misfolded SOD1 transferred through exosome-dependent and exosome-independent pathways Published In Cellular and Molecular Neurobiology
  2016	Protein aggregates stimulate macropinocytosis facilitating their propagation Published In Prion
  2016	Rapid flow cytometric measurement of protein inclusions and nuclear trafficking Published In Scientific Reports
  2016	Susceptibility of mutant SOD1 to form a destabilized monomer predicts cellular aggregation and toxicity but not in vitro aggregation propensity Published In Frontiers in Neuroscience
  2016	Walking the tightrope: proteostasis and neurodegenerative disease Published In Journal of Neurochemistry
  2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published In Scientific Reports
  2015	Evaluation of skin fibroblasts from amyotrophic lateral sclerosis patients for the rapid study of pathological features Published In Neurotoxicity Research
  2015	Expansion of suppressor tregs by IL-2/IL-2 MAB complexes slows disease progression in the mutant SOD1 mouse model of ALS Published In Journal of Neurochemistry
  2015	SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation Published In Molecular Neurodegeneration
  2015	SerpinB2 (PAI-2) modulates proteostasis via binding misfolded proteins and promotion of cytoprotective inclusion formation Published In PLoS One
  2015	The role of macropinocytosis in the propagation of protein aggregation associated with neurodegenerative diseases Published In Frontiers in Physiology
  2014	ALS-associated mutants of SOD1, TDP-43, FUS, C9ORF72 and UBQLN2 disrupt proteostasis in neuronal cell culture Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  2014	Amilial amyotrophic lateral sclerosis-associated proteins form aggregates via distinct pathways in cells Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  2014	Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms Published In Proceedings of the National Academy of Sciences of USA
  2014	Misfolded polyglutamine, polyalanine, and superoxide dismutase 1 aggregate via distinct pathways in the cell Published In Journal of Biological Chemistry
  2013	Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype Published In Glia
  2013	Extracellular chaperones Published In Topics in Current Chemistry
  2013	Extracellular chaperones and proteostasis Published In Annual Review of Biochemistry
  2013	Extracellular wildtype and mutant SOD1 induces ER-Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells Published In Cellular and Molecular Life Sciences
  2013	Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis Published In Scientific Reports
  2013	Intercellular propagated misfolding of wild-type Cu-Zn superoxide dismutase Published In Prion
  2013	P2X7 receptor activation induces reactive oxygen species formation and cell death in murine EOC13 microglia Published In Mediators of Inflammation
  2013	Protease-activated alpha-2-macroglobulin can inhibit amyloid formation via two distinct mechanisms Published In FEBS Letters
  2013	The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro Published In Cell Stress and Chaperones
  2012	Roles of extracellular chaperones in amyloidosis Published In Journal of Molecular Biology
  2011	Clusterin facilitates in vivo clearance of extracellular misfolded proteins Published In Cellular and Molecular Life Sciences
  2010	ANS binding reveals common features of cytotoxic amyloid species Published In ACS Chemical Biology
  2010	Extracellular chaperones modulate the effects of Alzheimer's patient cerebrospinal fluid on A beta(1-42) toxicity and uptake Published In Cell Stress and Chaperones
  2010	Protein chemistry of amyloid fibrils and chaperones: implications for amyloid formation and disease Published In Current Chemical Biology
  2010	The N-terminus of amyloid-beta plays a crucial role in its aggregation and toxicity Published In The Federation of European Biochemical Societies (FEBS) Journal
  2009	Macroglobulin and haptoglobin suppress amyloid formation by interacting with prefibrillar protein species Published In Journal of Biological Chemistry
  2009	Structural characterization of clusterin-chaperone client protein complexes Published In Journal of Biological Chemistry
  2009	Therapeutic targets in extracellular protein deposition diseases Published In Current Medicinal Chemistry
  2008	Clusterin interacts with paclitaxel and confer paclitaxel resistance in ovarian cancer Published In Neoplasia
  2008	Protease activation of α2-Macroglobulin modulates a chaperone-like action with broad specificity Published In Biochemistry
  2008	Think outside the cell Published In Chemical Biology
  2007	Potential roles of abundant extracellular chaperones in the control of amyloid formation and toxicity Published In Molecular Biosystems
  2007	The extracellular chaperone clusterin influences amyloid formation and toxicity by interacting with pre-fibrillar structures Published In The FASEB Journal
  2007	The extracellular chaperone clusterin potently inhibits human lysozyme amyloid formation by interacting with prefibrillar species Published In Journal of Molecular Biology

• Chapter

  Year	Title
  2021	Protein Aggregation in Amyotrophic Lateral Sclerosis Published In Spectrums of Amyotrophic Lateral Sclerosis Publisher Wiley
  2016	Network approaches to the understanding of Alzheimer’s disease: from model organisms to humans Published In Systems Biology of Alzheimer's Disease
  2015	The interplay of protein aggregates, microglia and neuroinflammation in neurodegenerative disease Published In Microglia: Physiology, Regulation and Health Implications
  2013	Extracellular chaperones Published In Molecular Chaperones Publisher Berlin
  2010	Extracellular Chaperones Publisher Springer Berlin Heidelberg
  2010	Extracellular Chaperones Publisher Springer Nature
  2010	Extracellular Chaperones. Published In Topics in current chemistry
  2009	The chaperone action of clusterin and its putative role in quality control of extracellular protein folding Published In Advances in cancer research Publisher San Diego, California, USA
  2008	Extracellular Chaperones and Amyloids Published In Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection Publisher United States

• Conference Paper

  Year	Title
  2019	Pathological spreading of TDP-43 studied in real time: Impaired microglia function leads to propagation of TDP-43 along the axon and into surrounding tissue Published In BRAIN PATHOLOGY
  2017	Ubiquitin proteasome system dysfunction in ALS patient IPSC-derived motor neurons Published In JOURNAL OF NEUROCHEMISTRY
  2016	Identification Of A novel FTD-MND gene on chromosome 16 Published In JOURNAL OF NEUROCHEMISTRY
  2016	The P2X7 receptor in amyotrophic lateral sclerosis Published In EUROPEAN JOURNAL OF IMMUNOLOGY
  2010	The N-terminus of amyloid-beta plays a crucial role in its aggregation and toxicity Published In FEBS JOURNAL

Investigator On

Year	Title
2022 - 2025	Targeting misfolded proteins with MisfoldUbLs as a therapeutic strategy for ALS Awarded by: FightMND Funding Scheme: Drug Discovery Grants
2021 - 2025	Using Proteostasis framework to understand and develop therapies for MND Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Investigator Grants
2022 - 2024	Enhancing delivery of gene therapy to motor neurons and glial cells using focused ultrasound Awarded by: FightMND Funding Scheme: Impact Research Grants
2020 - 2023	Combination therapy to improve on CuATSM outcomes in ALS Awarded by: FightMND Funding Scheme: Drug Discovery Grants
2022	How do protein quality control mechanisms maintain neural circuit function? Awarded by: Australian Research Council (ARC) Funding Scheme: Discovery Projects
2020 - 2022	Prion-like strains of TDP-43 aggregates in MND and FTLD Awarded by: FightMND Funding Scheme: Impact Research Grants
2020	Non-invasive drug delivery across the blood brain barrier: Improving the bioavailability of drugs for MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
2016 - 2020	Developing insight into the molecular origins of familial and sporadic frontotemporal dementia and amyotrophic lateral sclerosis Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Dementia Research Team Grants
2018 - 2019	Proteome homeostasis dysfunction in axon terminals as a cause of MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Betty Laidlaw MND Research Prize
2016 - 2019	The role of mutant cyclin F in amyotrophic lateral sclerosis Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Project Grant
2018	Astrocytic modulation of neuronal excitability and cell death in Motor Neurone Disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2017 - 2018	Establishing the therapeutic potential of the P2X7 receptor ion channel in amyotrophic lateral sclerosis Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2016 - 2018	Delivery of ubiquitin to motor neurons using a targeted, sterically stabilized liposome delivery system Awarded by: U.S. Department of Defense Funding Scheme: Congressionally Directed Medical Research Program
2016 - 2017	Development of a biocompatible functionalised liposome drug delivery system to increase efficiency of delivery to motor neurons Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2014	COUNTESS 11 FL Cell Counter and Qubit Quant Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2014	Examining the role of protein degradation in iPS cell models of ALS Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2013	Neon Transfection System: Workstation for transfer of genetic material into hard-to-work-with cells Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2012	Integrated facility for confocal imaging and single molecule flourescence analysis Awarded by: Australian Research Council (ARC) Funding Scheme: Linkage Infrastructure Equipment & Facilities (LIEF)
2012	Protein aggregation and chaperones: key players in MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2012	Ratk ERPM4 platform rocker etc. Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2011	Eppendorf MasterCycler Pro thermocycler Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2011	Protein Workstation Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2010 - 2011	Molecular chaperones as agents of the aging process Awarded by: IRT Research Foundation Funding Scheme: Grant
2009	Probing molecular mechanisms of microglial and astrocyte activation in ALS Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Bill Gole MND Postdoctoral Fellowship
2008	Attend 19 International Symposium on ALS/MND Birmingham Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Travel Grant
2008	The effects of alpha-2-macroglobulin on amyloid formation and toxicity Awarded by: Australian Research Council (ARC) Funding Scheme: Linkage International
2007	Do the effects of extracellular chaperones on A beta clearance and toxicity provide potential therapeutic targets? Awarded by: Alzheimer's Australia Research (AAR) Funding Scheme: Dementia Grants Program
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	Barnes Mazes for rats and mice Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Contributions to Leica Dmi8 microscope Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Does the transfer of ALS protein aggregates between motor neurons trigger neurodegeneration? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
	GoBlot machines and Leica Objective Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Is UBA1 a therapeutic target in ALS? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
	Lumenera Infinity 2-5 microscope colour camera Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Rapid screening on small molecule libraries to identify new drug leads to treat Motor Neurone Disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
	Ubiquitin Proteasome System dysfunction as a biomarker for the diagnosis and prognosis of motor neuron disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
	Ubiquitin depletion as a cause of Amyotrophic Lateral Schlerosis (ALS) Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid

Impact Story

Advisees

• Graduate Advising Relationship

  Degree	Research Title	Advisee
  Doctor of Philosophy	Investigating the role of proteostasis pathways in regulating the intracellular inclusion formation of firefly luciferase: a model system to study protein aggregation in cells	McMahon, Shannon
  Doctor of Philosophy	The Role of Ubiquitin Homeostasis in ALS	Chisholm, Christen
  Doctor of Philosophy	Is FUS a prion in ALS	Walker, Tom

Organizer Of Event

• Proteostasis and Disease Symposium 2012 -

Keywords

• Motor Neurone Disease 
  Amyotrophic Lateral Sclerosis 
  Protein
  Proteostasis
  
  

Orcid Id

• http://orcid.org/0000-0003-2528-7039 (pending confirmation)

Scopus Id

•  9739159000

Top Publications

Year	Title
2017	Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS Published in   Proceedings of the National Academy of Sciences of USA
2014	Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms Published in   Proceedings of the National Academy of Sciences of USA
2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published in   Scientific Reports
2016	CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia Published in   Nature Communications

Research Areas

• 0601 - BIOCHEMISTRY AND CELL BIOLOGY
• 1109 - NEUROSCIENCES
• Motor (UMLS)
• protein (UMLS)
• protein (UMLS)

Research Overview

• The Yerbury Lab focuses on the understanding of the molecular processes underpinning the Motor Neurone Disease (MND) Amyotrophic Lateral Sclerosis (ALS) with a particular emphasis on protein misfolding, protein aggregation and inclusion formation. We think that by understanding the process of protein aggregation in the cellular environment that we might be able to uncover why neurons die in motor neurone disease. Our team is part of the Proteostasis and Disease Research Centre at the University of Wollongong and is housed in the Illawarra Health and Medical Research Institute.

Member Of

• Proteostasis and Disease Research Centre Member 2012 -
• Illawarra Health and Medical Research Institute Member 2011 -
• Centre for Medical and Molecular Bioscience 2010 -

Has Collaborator

• Ecroyd, Heath  Available as Research Supervisor
• Ooi, Lezanne  Professor
• Perrow, Kara  Dr
• Ranson, Marie  Professor
• Sluyter, Ronald  Faculty Academic
• Wilson, Mark  Senior Professor

Selected Publications

• Journal Article

  Year	Title
  2022	A copper chaperone–mimetic polytherapy for SOD1-associated amyotrophic lateral sclerosis Published In Journal of Biological Chemistry
  2022	Cells Overexpressing ALS-Associated SOD1 Variants Are Differentially Susceptible to CuATSM-Associated Toxicity Published In ACS Chemical Neuroscience
  2022	P2X7 receptor activation mediates superoxide dismutase 1 (SOD1) release from murine NSC-34 motor neurons Published In Purinergic Signalling
  2022	Proteostasis impairment and ALS Published In Progress in Biophysics and Molecular Biology
  2022	TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex Published In Neurobiology of Disease
  2022	Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateral sclerosis? Published In Neural Regeneration Research
  2022	Vulnerability of the spinal motor neuron presynaptic terminal sub-proteome in ALS Published In Neuroscience Letters
  2021	Behavioural effects of cage systems on the G93A Superoxide Dismutase 1 transgenic mouse model for amyotrophic lateral sclerosis Published In Genes, Brain and Behavior
  2021	CuATSM improves motor function and extends survival but is not tolerated at a high dose in SOD1 G93A mice with a C57BL/6 background Published In Scientific Reports
  2021	Disabled in academia: to be or not to be, that is the question Published In Trends in Neurosciences
  2021	Mutant Cu/Zn Superoxide Dismutase (A4V) Turnover Is Altered in Cells Containing Inclusions Published In Frontiers in Molecular Neuroscience
  2021	Non-Viral Vector-Mediated Gene Therapy for ALS: Challenges and Future Perspectives Published In Molecular Pharmaceutics
  2021	Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations in CCNF Reveals Activation of the Apoptosis Pathway: A Workflow to Screen Pathogenic Gene Mutations Published In Frontiers in Molecular Neuroscience
  2021	p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death Published In Scientific Reports
  2020	Amyotrophic Lateral Sclerosis: Proteins, Proteostasis, Prions, and Promises Published In Frontiers in Cellular Neuroscience
  2020	Corrigendum: Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis (Frontiers in Molecular Neuroscience, (2019), 12, 10.3389/fnmol.2019.00262) Published In Frontiers in Molecular Neuroscience
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells Published In Journal of cell science
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells. Published In J Cell Sci
  2020	Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal-like cells
  2020	Novel behavioural characteristics of the superoxide dismutase 1 G93A (SOD1G93A) mouse model of amyotrophic lateral sclerosis include sex-dependent phenotypes Published In Genes, Brain and Behavior
  2020	Proteome Homeostasis Dysfunction: A Unifying Principle in ALS Pathogenesis Published In Trends in Neurosciences
  2020	Selenium-based compounds: Emerging players in the ever-unfolding story of SOD1 in amyotrophic lateral sclerosis. Published In EBioMedicine
  2020	The P2X7 receptor antagonist JNJ-47965567 administered thrice weekly from disease onset does not alter progression of amyotrophic lateral sclerosis in SOD1G93A mice Published In Purinergic Signalling
  2020	The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins Published In Glia
  2020	The pivotal role of ubiquitin-activating enzyme E1 (UBA1) in neuronal health and neurodegeneration Published In International Journal of Biochemistry and Cell Biology
  2020	The prion-like nature of amyotrophic lateral sclerosis
  2020	Trajectory Taken by Dimeric Cu/Zn Superoxide Dismutase through the Protein Unfolding and Dissociation Landscape Is Modulated by Salt Bridge Formation Published In Analytical Chemistry
  2020	Tryptophan residue 32 in human Cu-Zn superoxide dismutase modulates prion-like propagation and strain selection Published In PLoS One
  2020	Ubiquitin Homeostasis Is Disrupted in TDP-43 and FUS Cell Models of ALS Published In iScience
  2019	Dynamic interplay between H-current and M-current controls motoneuron hyperexcitability in amyotrophic lateral sclerosis Published In Cell Death and Disease
  2019	Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis Published In Frontiers in Molecular Neuroscience
  2019	Strategies to promote the maturation of ALS-associated SOD1 mutants: Small molecules return to the fold Published In Neural Regeneration Research
  2019	The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation Published In Cells
  2019	The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS Published In Neuroscience Letters
  2019	Using Tetracysteine-Tagged TDP-43 with a Biarsenical Dye To Monitor Real-Time Trafficking in a Cell Model of Amyotrophic Lateral Sclerosis Published In Biochemistry
  2018	Assessment of metal concentrations in the SOD1G93Amouse model of amyotrophic lateral sclerosis and its potential role in muscular denervation, with particular focus on muscle tissue Published In Molecular and Cellular Neuroscience
  2018	Association of Regulatory T-Cell Expansion With Progression of Amyotrophic Lateral Sclerosis: A Study of Humans and a Transgenic Mouse Model Published In Jama Neurology
  2018	CuATSM Protects Against the in Vitro Cytotoxicity of Wild-Type-Like Copper-Zinc Superoxide Dismutase Mutants but not Mutants That Disrupt Metal Binding Published In ACS Chemical Neuroscience
  2018	Dysregulation of microRNA biogenesis machinery and microRNA/RNA ratio in skeletal muscle of amyotrophic lateral sclerosis mice Published In Muscle and Nerve
  2018	Nucleo-cytoplasmic transport of TDP-43 studied in real time: impaired microglia function leads to axonal spreading of TDP-43 in degenerating motor neurons Published In Acta Neuropathologica
  2018	Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48‑linked ubiquitylation and defective autophagy Published In Cellular and Molecular Life Sciences
  2018	SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS Published In Journal of Cell Science
  2018	Some diseases, like mine, deteriorate rapidly – disability services need to keep up Published In The Conversation
  2018	The cysteine-reactive small molecule ebselen facilitates effective SOD1 maturation Published In Nature Communications
  2018	Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells Published In Scientific Reports
  2017	Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation Published In Cell Stress and Chaperones
  2017	Casein kinase II phosphorylation of cyclin F at serine 621 regulates the Lys48-ubiquitylation E3 ligase activity of the SCF(cyclin F) complex Published In Open Biology
  2017	Clusterin protects neurons against intracellular proteotoxicity Published In Acta Neuropathologica Communications
  2017	Flow cytometric measurement of the cellular propagation of TDP-43 aggregation Published In Prion
  2017	Improving the Delivery of SOD1 Antisense Oligonucleotides to Motor Neurons Using Calcium Phosphate-Lipid Nanoparticles Published In Frontiers in Neuroscience
  2017	Longitudinal assessment of metal concentrations and copper isotope ratios in the G93A SOD1 mouse model of amyotrophic lateral sclerosis Published In Metallomics: integrated biometal science
  2017	Motor neuron disease proteins activate complement and generate C5a Published In MOLECULAR IMMUNOLOGY
  2017	P2X7 antagonism using Brilliant Blue G reduces body weight loss and prolongs survival in female SOD1G93A amyotrophic lateral sclerosis mice Published In PeerJ
  2017	P2X7 receptor antagonism in amyotrophic lateral sclerosis Published In Neural Regeneration Research
  2017	Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS Published In Proceedings of the National Academy of Sciences of USA
  2017	The heat shock response in neurons and astroglia and its role in neurodegenerative diseases Published In Molecular Neurodegeneration
  2017	The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis Published In BBA: Molecular Basis of Disease
  2016	CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia Published In Nature Communications
  2016	Disease mechanisms in ALS: misfolded SOD1 transferred through exosome-dependent and exosome-independent pathways Published In Cellular and Molecular Neurobiology
  2016	Protein aggregates stimulate macropinocytosis facilitating their propagation Published In Prion
  2016	Rapid flow cytometric measurement of protein inclusions and nuclear trafficking Published In Scientific Reports
  2016	Susceptibility of mutant SOD1 to form a destabilized monomer predicts cellular aggregation and toxicity but not in vitro aggregation propensity Published In Frontiers in Neuroscience
  2016	Walking the tightrope: proteostasis and neurodegenerative disease Published In Journal of Neurochemistry
  2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published In Scientific Reports
  2015	Evaluation of skin fibroblasts from amyotrophic lateral sclerosis patients for the rapid study of pathological features Published In Neurotoxicity Research
  2015	Expansion of suppressor tregs by IL-2/IL-2 MAB complexes slows disease progression in the mutant SOD1 mouse model of ALS Published In Journal of Neurochemistry
  2015	SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation Published In Molecular Neurodegeneration
  2015	SerpinB2 (PAI-2) modulates proteostasis via binding misfolded proteins and promotion of cytoprotective inclusion formation Published In PLoS One
  2015	The role of macropinocytosis in the propagation of protein aggregation associated with neurodegenerative diseases Published In Frontiers in Physiology
  2014	ALS-associated mutants of SOD1, TDP-43, FUS, C9ORF72 and UBQLN2 disrupt proteostasis in neuronal cell culture Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  2014	Amilial amyotrophic lateral sclerosis-associated proteins form aggregates via distinct pathways in cells Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  2014	Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms Published In Proceedings of the National Academy of Sciences of USA
  2014	Misfolded polyglutamine, polyalanine, and superoxide dismutase 1 aggregate via distinct pathways in the cell Published In Journal of Biological Chemistry
  2013	Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype Published In Glia
  2013	Extracellular chaperones Published In Topics in Current Chemistry
  2013	Extracellular chaperones and proteostasis Published In Annual Review of Biochemistry
  2013	Extracellular wildtype and mutant SOD1 induces ER-Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells Published In Cellular and Molecular Life Sciences
  2013	Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis Published In Scientific Reports
  2013	Intercellular propagated misfolding of wild-type Cu-Zn superoxide dismutase Published In Prion
  2013	P2X7 receptor activation induces reactive oxygen species formation and cell death in murine EOC13 microglia Published In Mediators of Inflammation
  2013	Protease-activated alpha-2-macroglobulin can inhibit amyloid formation via two distinct mechanisms Published In FEBS Letters
  2013	The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro Published In Cell Stress and Chaperones
  2012	Roles of extracellular chaperones in amyloidosis Published In Journal of Molecular Biology
  2011	Clusterin facilitates in vivo clearance of extracellular misfolded proteins Published In Cellular and Molecular Life Sciences
  2010	ANS binding reveals common features of cytotoxic amyloid species Published In ACS Chemical Biology
  2010	Extracellular chaperones modulate the effects of Alzheimer's patient cerebrospinal fluid on A beta(1-42) toxicity and uptake Published In Cell Stress and Chaperones
  2010	Protein chemistry of amyloid fibrils and chaperones: implications for amyloid formation and disease Published In Current Chemical Biology
  2010	The N-terminus of amyloid-beta plays a crucial role in its aggregation and toxicity Published In The Federation of European Biochemical Societies (FEBS) Journal
  2009	Macroglobulin and haptoglobin suppress amyloid formation by interacting with prefibrillar protein species Published In Journal of Biological Chemistry
  2009	Structural characterization of clusterin-chaperone client protein complexes Published In Journal of Biological Chemistry
  2009	Therapeutic targets in extracellular protein deposition diseases Published In Current Medicinal Chemistry
  2008	Clusterin interacts with paclitaxel and confer paclitaxel resistance in ovarian cancer Published In Neoplasia
  2008	Protease activation of α2-Macroglobulin modulates a chaperone-like action with broad specificity Published In Biochemistry
  2008	Think outside the cell Published In Chemical Biology
  2007	Potential roles of abundant extracellular chaperones in the control of amyloid formation and toxicity Published In Molecular Biosystems
  2007	The extracellular chaperone clusterin influences amyloid formation and toxicity by interacting with pre-fibrillar structures Published In The FASEB Journal
  2007	The extracellular chaperone clusterin potently inhibits human lysozyme amyloid formation by interacting with prefibrillar species Published In Journal of Molecular Biology

• Chapter

  Year	Title
  2021	Protein Aggregation in Amyotrophic Lateral Sclerosis Published In Spectrums of Amyotrophic Lateral Sclerosis Publisher Wiley
  2016	Network approaches to the understanding of Alzheimer’s disease: from model organisms to humans Published In Systems Biology of Alzheimer's Disease
  2015	The interplay of protein aggregates, microglia and neuroinflammation in neurodegenerative disease Published In Microglia: Physiology, Regulation and Health Implications
  2013	Extracellular chaperones Published In Molecular Chaperones Publisher Berlin
  2010	Extracellular Chaperones Publisher Springer Berlin Heidelberg
  2010	Extracellular Chaperones Publisher Springer Nature
  2010	Extracellular Chaperones. Published In Topics in current chemistry
  2009	The chaperone action of clusterin and its putative role in quality control of extracellular protein folding Published In Advances in cancer research Publisher San Diego, California, USA
  2008	Extracellular Chaperones and Amyloids Published In Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection Publisher United States

• Conference Paper

  Year	Title
  2019	Pathological spreading of TDP-43 studied in real time: Impaired microglia function leads to propagation of TDP-43 along the axon and into surrounding tissue Published In BRAIN PATHOLOGY
  2017	Ubiquitin proteasome system dysfunction in ALS patient IPSC-derived motor neurons Published In JOURNAL OF NEUROCHEMISTRY
  2016	Identification Of A novel FTD-MND gene on chromosome 16 Published In JOURNAL OF NEUROCHEMISTRY
  2016	The P2X7 receptor in amyotrophic lateral sclerosis Published In EUROPEAN JOURNAL OF IMMUNOLOGY
  2010	The N-terminus of amyloid-beta plays a crucial role in its aggregation and toxicity Published In FEBS JOURNAL

Investigator On

Year	Title
2022 - 2025	Targeting misfolded proteins with MisfoldUbLs as a therapeutic strategy for ALS Awarded by: FightMND Funding Scheme: Drug Discovery Grants
2021 - 2025	Using Proteostasis framework to understand and develop therapies for MND Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Investigator Grants
2022 - 2024	Enhancing delivery of gene therapy to motor neurons and glial cells using focused ultrasound Awarded by: FightMND Funding Scheme: Impact Research Grants
2020 - 2023	Combination therapy to improve on CuATSM outcomes in ALS Awarded by: FightMND Funding Scheme: Drug Discovery Grants
2022	How do protein quality control mechanisms maintain neural circuit function? Awarded by: Australian Research Council (ARC) Funding Scheme: Discovery Projects
2020 - 2022	Prion-like strains of TDP-43 aggregates in MND and FTLD Awarded by: FightMND Funding Scheme: Impact Research Grants
2020	Non-invasive drug delivery across the blood brain barrier: Improving the bioavailability of drugs for MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
2016 - 2020	Developing insight into the molecular origins of familial and sporadic frontotemporal dementia and amyotrophic lateral sclerosis Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Dementia Research Team Grants
2018 - 2019	Proteome homeostasis dysfunction in axon terminals as a cause of MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Betty Laidlaw MND Research Prize
2016 - 2019	The role of mutant cyclin F in amyotrophic lateral sclerosis Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Project Grant
2018	Astrocytic modulation of neuronal excitability and cell death in Motor Neurone Disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2017 - 2018	Establishing the therapeutic potential of the P2X7 receptor ion channel in amyotrophic lateral sclerosis Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2016 - 2018	Delivery of ubiquitin to motor neurons using a targeted, sterically stabilized liposome delivery system Awarded by: U.S. Department of Defense Funding Scheme: Congressionally Directed Medical Research Program
2016 - 2017	Development of a biocompatible functionalised liposome drug delivery system to increase efficiency of delivery to motor neurons Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2014	COUNTESS 11 FL Cell Counter and Qubit Quant Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2014	Examining the role of protein degradation in iPS cell models of ALS Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2013	Neon Transfection System: Workstation for transfer of genetic material into hard-to-work-with cells Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2012	Integrated facility for confocal imaging and single molecule flourescence analysis Awarded by: Australian Research Council (ARC) Funding Scheme: Linkage Infrastructure Equipment & Facilities (LIEF)
2012	Protein aggregation and chaperones: key players in MND Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2012	Ratk ERPM4 platform rocker etc. Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2011	Eppendorf MasterCycler Pro thermocycler Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2011	Protein Workstation Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2010 - 2011	Molecular chaperones as agents of the aging process Awarded by: IRT Research Foundation Funding Scheme: Grant
2009	Probing molecular mechanisms of microglial and astrocyte activation in ALS Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Bill Gole MND Postdoctoral Fellowship
2008	Attend 19 International Symposium on ALS/MND Birmingham Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Travel Grant
2008	The effects of alpha-2-macroglobulin on amyloid formation and toxicity Awarded by: Australian Research Council (ARC) Funding Scheme: Linkage International
2007	Do the effects of extracellular chaperones on A beta clearance and toxicity provide potential therapeutic targets? Awarded by: Alzheimer's Australia Research (AAR) Funding Scheme: Dementia Grants Program
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	Barnes Mazes for rats and mice Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Contributions to Leica Dmi8 microscope Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Does the transfer of ALS protein aggregates between motor neurons trigger neurodegeneration? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
	GoBlot machines and Leica Objective Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Is UBA1 a therapeutic target in ALS? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
	Lumenera Infinity 2-5 microscope colour camera Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
	Rapid screening on small molecule libraries to identify new drug leads to treat Motor Neurone Disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Innovator Grant
	Ubiquitin Proteasome System dysfunction as a biomarker for the diagnosis and prognosis of motor neuron disease Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
	Ubiquitin depletion as a cause of Amyotrophic Lateral Schlerosis (ALS) Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid

Impact Story

Advisees

• Graduate Advising Relationship

  Degree	Research Title	Advisee
  Doctor of Philosophy	Investigating the role of proteostasis pathways in regulating the intracellular inclusion formation of firefly luciferase: a model system to study protein aggregation in cells	McMahon, Shannon
  Doctor of Philosophy	The Role of Ubiquitin Homeostasis in ALS	Chisholm, Christen
  Doctor of Philosophy	Is FUS a prion in ALS	Walker, Tom

Organizer Of Event

• Proteostasis and Disease Symposium 2012 -

Keywords

• Motor Neurone Disease 
  Amyotrophic Lateral Sclerosis 
  Protein
  Proteostasis
  
  

Orcid Id

• http://orcid.org/0000-0003-2528-7039 (pending confirmation)

Scopus Id

•  9739159000