Yerbury, Justin J.

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

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.

Available for Collaborative Projects

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
Mark R Wilson Senior Professor
Ooi, Lezanne Associate Professor
Ranson, Marie Professor
Sluyter, Ronald Faculty Academic
Vine, Kara L. Dr

Selected Publications

Journal Article

Year	Title
2019	Dynamic interplay between H-current and M-current controls motoneuron hyperexcitability in amyotrophic lateral sclerosis Published In Cell Death and Disease Volume 10 Pages 310-1 - 310-13 ISSN 2041-4889 Altmetric
2019	Strategies to promote the maturation of ALS-associated SOD1 mutants: Small molecules return to the fold Published In Neural Regeneration Research Volume 14 Pages 1511 - 1512 Altmetric
2019	The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation Published In Cells Volume 8 Pages 581-1 - 581-17 Altmetric
2019	The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS Published In Neuroscience Letters Volume 704 Pages 89 - 94 ISSN 0304-3940 Altmetric
2018	Assessment of metal concentrations in the SOD1^G93Amouse model of amyotrophic lateral sclerosis and its potential role in muscular denervation, with particular focus on muscle tissue Published In Molecular and Cellular Neuroscience Volume 88 Pages 319 - 329 ISSN 1044-7431 Altmetric
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 Volume 75 Pages 681 - 689 Altmetric
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 Volume Online First Pages 1 - 10 ISSN 1948-7193 Altmetric
2018	Dysregulation of microRNA biogenesis machinery and microRNA/RNA ratio in skeletal muscle of amyotrophic lateral sclerosis mice Published In Muscle and Nerve Volume 57 Pages 838 - 847 ISSN 0148-639X Altmetric
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 Volume 136 Pages 445 - 459 ISSN 0001-6322 Altmetric
2018	Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48‑linked ubiquitylation and defective autophagy Published In Cellular and Molecular Life Sciences Volume 75 Pages 335 - 354 ISSN 1420-682X Altmetric
2018	SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS Published In Journal of Cell Science Volume 131 Pages jcs209122-1 - jcs209122-14 ISSN 0021-9533 Altmetric
2018	Some diseases, like mine, deteriorate rapidly – disability services need to keep up Published In The Conversation Volume 9 November Pages 1 - 3
2018	The cysteine-reactive small molecule ebselen facilitates effective SOD1 maturation Published In Nature Communications Volume 9 Pages 1693-1 - 1693-9 ISSN 2041-1723 Altmetric
2018	Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells Published In Scientific Reports Volume 8 Pages 15590-1 - 15590-12 ISSN 2045-2322 Altmetric
2017	Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation Published In Cell Stress and Chaperones Volume 22 Pages 893 - 902 ISSN 1355-8145 Altmetric
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 Volume 7 Pages 170058-1 - 170058-11 ISSN 2046-2441 Altmetric
2017	Clusterin protects neurons against intracellular proteotoxicity Published In Acta Neuropathologica Communications Volume 5 Pages 81-1 - 81-16 Altmetric
2017	Flow cytometric measurement of the cellular propagation of TDP-43 aggregation Published In Prion Volume 11 Pages 195 - 204 ISSN 1933-6896 Altmetric
2017	Improving the Delivery of SOD1 Antisense Oligonucleotides to Motor Neurons Using Calcium Phosphate-Lipid Nanoparticles Published In Frontiers in Neuroscience Volume 11 Pages 476-1 - 476-12 ISSN 1662-453X Altmetric
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 Volume 9 Pages 161 - 174 ISSN 1756-5901 Altmetric
2017	P2X7 antagonism using Brilliant Blue G reduces body weight loss and prolongs survival in female SOD1G93A amyotrophic lateral sclerosis mice Published In PeerJ Volume 5 Pages e3064-1 - e3064-29 Altmetric
2017	P2X7 receptor antagonism in amyotrophic lateral sclerosis Published In Neural Regeneration Research Volume 12 Pages 749 - 750 Altmetric
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 Volume 114 Pages E3935 - E3943 ISSN 1091-6490 Altmetric
2017	The heat shock response in neurons and astroglia and its role in neurodegenerative diseases Published In Molecular Neurodegeneration Volume 12 Pages 65-1 - 65-20 ISSN 1750-1326 Altmetric
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 Volume 1863 Pages 2762 - 2771 ISSN 0925-4439 Altmetric
2016	CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia Published In Nature Communications Volume 7 Pages 11253-1 - 11253-8 ISSN 2041-1723 Altmetric
2016	Disease mechanisms in ALS: misfolded SOD1 transferred through exosome-dependent and exosome-independent pathways Published In Cellular and Molecular Neurobiology Volume 36 Pages 377 - 381 ISSN 0272-4340 Altmetric
2016	Protein aggregates stimulate macropinocytosis facilitating their propagation Published In Prion Volume 10 Pages 119 - 126 ISSN 1933-6896 Altmetric
2016	Rapid flow cytometric measurement of protein inclusions and nuclear trafficking Published In Scientific Reports Volume 6 Pages 31138-1 - 31138-9 ISSN 2045-2322 Altmetric
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 Volume 10 Pages 499-1 - 499-16 ISSN 1662-453X Altmetric
2016	Walking the tightrope: proteostasis and neurodegenerative disease Published In Journal of Neurochemistry Volume 137 Pages 489 - 505 ISSN 0022-3042 Altmetric
2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published In Scientific Reports Volume 5 Pages 13416-1 - 13416-14 ISSN 2045-2322 Altmetric
2015	Evaluation of skin fibroblasts from amyotrophic lateral sclerosis patients for the rapid study of pathological features Published In Neurotoxicity Research Volume 28 Pages 138 - 146 ISSN 1029-8428 Altmetric
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 Volume 134 Pages 206 - 207 ISSN 0022-3042 Altmetric
2015	SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation Published In Molecular Neurodegeneration Volume 10 Pages 57-1 - 57-17 ISSN 1750-1326 Altmetric
2015	SerpinB2 (PAI-2) modulates proteostasis via binding misfolded proteins and promotion of cytoprotective inclusion formation Published In PLoS One Volume 10 Pages e0130136-1 - e0130136-20 ISSN 1932-6203 Altmetric
2015	The role of macropinocytosis in the propagation of protein aggregation associated with neurodegenerative diseases Published In Frontiers in Physiology Volume 6 Pages 277-1 - 277-8 ISSN 1664-042X Altmetric
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 Volume 15 Pages 170 - 171 Altmetric
2014	Amilial amyotrophic lateral sclerosis-associated proteins form aggregates via distinct pathways in cells Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration Volume 15 Pages 170 - 170 Altmetric
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 Volume 111 Pages 3620 - 3625 ISSN 1091-6490 Altmetric
2014	Misfolded polyglutamine, polyalanine, and superoxide dismutase 1 aggregate via distinct pathways in the cell Published In Journal of Biological Chemistry Volume 289 Pages 6669 - 6680 ISSN 0021-9258 Altmetric
2013	Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype Published In Glia Volume 61 Pages 409 - 419 ISSN 0894-1491 Altmetric
2013	Extracellular chaperones and proteostasis Published In Annual Review of Biochemistry Volume 82 Pages 295 - 322 ISSN 0066-4154 Altmetric
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 Volume 70 Pages 4181 - 4195 ISSN 1420-682X Altmetric
2013	Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis Published In Scientific Reports Volume 3 Pages 3275-1 - 3275-6 ISSN 2045-2322 Altmetric
2013	Intercellular propagated misfolding of wild-type Cu-Zn superoxide dismutase Published In Prion Volume 7 Pages 76 - 76 ISSN 1933-6896
2013	P2X7 receptor activation induces reactive oxygen species formation and cell death in murine EOC13 microglia Published In Mediators of Inflammation Volume 2013 Pages 271813-1 - 271813-18 ISSN 0962-9351 Altmetric
2013	Protease-activated alpha-2-macroglobulin can inhibit amyloid formation via two distinct mechanisms Published In FEBS Letters Volume 587 Pages 398 - 403 ISSN 0014-5793 Altmetric
2013	The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro Published In Cell Stress and Chaperones Volume 18 Pages 251 - 257 ISSN 1355-8145 Altmetric
2012	Roles of extracellular chaperones in amyloidosis Published In Journal of Molecular Biology Volume 421 Pages 499 - 516 ISSN 0022-2836 Altmetric
2011	Clusterin facilitates in vivo clearance of extracellular misfolded proteins Published In Cellular and Molecular Life Sciences Volume 68 Pages 3919 - 3931 ISSN 1420-682X Altmetric
2010	ANS binding reveals common features of cytotoxic amyloid species Published In ACS Chemical Biology Volume 5 Pages 735 - 740 ISSN 1554-8929 Altmetric
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 Volume 15 Pages 115 - 121 ISSN 1355-8145 Altmetric
2010	Protein chemistry of amyloid fibrils and chaperones: implications for amyloid formation and disease Published In Current Chemical Biology Volume 4 Pages 89 - 98 Altmetric
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 Volume 277 Pages 79 - 80 ISSN 1742-464X Altmetric
2009	Macroglobulin and haptoglobin suppress amyloid formation by interacting with prefibrillar protein species Published In Journal of Biological Chemistry Volume 284 Pages 4246 - 4254 ISSN 0021-9258 Altmetric
2009	Structural characterization of clusterin-chaperone client protein complexes Published In Journal of Biological Chemistry Volume 284 Pages 21920 - 21927 ISSN 0021-9258 Altmetric
2009	Therapeutic targets in extracellular protein deposition diseases Published In Current Medicinal Chemistry Volume 16 Pages 2855 - 2866 ISSN 0929-8673 Altmetric
2008	Clusterin interacts with paclitaxel and confer paclitaxel resistance in ovarian cancer Published In Neoplasia Volume 10 Pages 964 - 972 ISSN 1522-8002
2008	Protease activation of α2-Macroglobulin modulates a chaperone-like action with broad specificity Published In Biochemistry Volume 47 Pages 1176 - 1185 ISSN 0006-2960 Altmetric
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 Volume 4 Pages 42 - 52 ISSN 1742-2051 Altmetric
2007	The extracellular chaperone clusterin influences amyloid formation and toxicity by interacting with pre-fibrillar structures Published In The FASEB Journal Volume 21 Pages 2312 - 2322 ISSN 0892-6638 Altmetric
2007	The extracellular chaperone clusterin potently inhibits human lysozyme amyloid formation by interacting with prefibrillar species Published In Journal of Molecular Biology Volume 369 Pages 157 - 167 ISSN 0022-2836 Altmetric

Chapter

Year	Title
2016	Network approaches to the understanding of Alzheimer’s disease: from model organisms to humans Published In Systems Biology of Alzheimer's Disease Publisher Humana Press Pages 447 - 458 Altmetric
2015	The interplay of protein aggregates, microglia and neuroinflammation in neurodegenerative disease Published In Microglia: Physiology, Regulation and Health Implications Publisher Nova science publishers Pages 157 - 204
2013	Extracellular chaperones Published In Molecular Chaperones Publisher Berlin: Springer Berlin Heidelberg Pages 241 - 268 Altmetric
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 : Elsevier Pages 89 - 114 Altmetric
2008	Extracellular Chaperones and Amyloids Published In Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection Publisher United States: Springer Science Pages 283 - 315 Altmetric

Investigator On

Year	Title
2019 - 2020	Is UBA1 a therapeutic target in ALS? 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
2019	GoBlot machines and Leica Objective Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2019	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
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
2017	Lumenera Infinity 2-5 microscope colour camera Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
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
2016	Contributions to Leica Dmi8 microscope Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2016	Does the transfer of ALS protein aggregates between motor neurons trigger neurodegeneration? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2016	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
2016	Ubiquitin depletion as a cause of Amyotrophic Lateral Schlerosis (ALS) 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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	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

Impact Story

Advisees

Graduate Advising Relationship

Degree	Research Title	Advisee
Doctor of Philosophy	Examination of the Ubiquitin Proteasome System in Induced Pluripotent Stem Cell Derived Motor Neurons, and Related Cell Types in the Context of Amyotrophic Lateral Sclerosis	Bax, Monique
Doctor of Philosophy	Investigating the role of proteostasis pathways in regulating the intracellular protein aggregation 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	Are proteostasis defects responsible for ALS.	Lambert-Smith, Isabella

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

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
Mark R Wilson Senior Professor
Ooi, Lezanne Associate Professor
Ranson, Marie Professor
Sluyter, Ronald Faculty Academic
Vine, Kara L. Dr

Selected Publications

Journal Article

Year	Title
2019	Dynamic interplay between H-current and M-current controls motoneuron hyperexcitability in amyotrophic lateral sclerosis Published In Cell Death and Disease Volume 10 Pages 310-1 - 310-13 ISSN 2041-4889 Altmetric
2019	Strategies to promote the maturation of ALS-associated SOD1 mutants: Small molecules return to the fold Published In Neural Regeneration Research Volume 14 Pages 1511 - 1512 Altmetric
2019	The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation Published In Cells Volume 8 Pages 581-1 - 581-17 Altmetric
2019	The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS Published In Neuroscience Letters Volume 704 Pages 89 - 94 ISSN 0304-3940 Altmetric
2018	Assessment of metal concentrations in the SOD1^G93Amouse model of amyotrophic lateral sclerosis and its potential role in muscular denervation, with particular focus on muscle tissue Published In Molecular and Cellular Neuroscience Volume 88 Pages 319 - 329 ISSN 1044-7431 Altmetric
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 Volume 75 Pages 681 - 689 Altmetric
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 Volume Online First Pages 1 - 10 ISSN 1948-7193 Altmetric
2018	Dysregulation of microRNA biogenesis machinery and microRNA/RNA ratio in skeletal muscle of amyotrophic lateral sclerosis mice Published In Muscle and Nerve Volume 57 Pages 838 - 847 ISSN 0148-639X Altmetric
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 Volume 136 Pages 445 - 459 ISSN 0001-6322 Altmetric
2018	Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48‑linked ubiquitylation and defective autophagy Published In Cellular and Molecular Life Sciences Volume 75 Pages 335 - 354 ISSN 1420-682X Altmetric
2018	SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS Published In Journal of Cell Science Volume 131 Pages jcs209122-1 - jcs209122-14 ISSN 0021-9533 Altmetric
2018	Some diseases, like mine, deteriorate rapidly – disability services need to keep up Published In The Conversation Volume 9 November Pages 1 - 3
2018	The cysteine-reactive small molecule ebselen facilitates effective SOD1 maturation Published In Nature Communications Volume 9 Pages 1693-1 - 1693-9 ISSN 2041-1723 Altmetric
2018	Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells Published In Scientific Reports Volume 8 Pages 15590-1 - 15590-12 ISSN 2045-2322 Altmetric
2017	Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation Published In Cell Stress and Chaperones Volume 22 Pages 893 - 902 ISSN 1355-8145 Altmetric
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 Volume 7 Pages 170058-1 - 170058-11 ISSN 2046-2441 Altmetric
2017	Clusterin protects neurons against intracellular proteotoxicity Published In Acta Neuropathologica Communications Volume 5 Pages 81-1 - 81-16 Altmetric
2017	Flow cytometric measurement of the cellular propagation of TDP-43 aggregation Published In Prion Volume 11 Pages 195 - 204 ISSN 1933-6896 Altmetric
2017	Improving the Delivery of SOD1 Antisense Oligonucleotides to Motor Neurons Using Calcium Phosphate-Lipid Nanoparticles Published In Frontiers in Neuroscience Volume 11 Pages 476-1 - 476-12 ISSN 1662-453X Altmetric
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 Volume 9 Pages 161 - 174 ISSN 1756-5901 Altmetric
2017	P2X7 antagonism using Brilliant Blue G reduces body weight loss and prolongs survival in female SOD1G93A amyotrophic lateral sclerosis mice Published In PeerJ Volume 5 Pages e3064-1 - e3064-29 Altmetric
2017	P2X7 receptor antagonism in amyotrophic lateral sclerosis Published In Neural Regeneration Research Volume 12 Pages 749 - 750 Altmetric
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 Volume 114 Pages E3935 - E3943 ISSN 1091-6490 Altmetric
2017	The heat shock response in neurons and astroglia and its role in neurodegenerative diseases Published In Molecular Neurodegeneration Volume 12 Pages 65-1 - 65-20 ISSN 1750-1326 Altmetric
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 Volume 1863 Pages 2762 - 2771 ISSN 0925-4439 Altmetric
2016	CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia Published In Nature Communications Volume 7 Pages 11253-1 - 11253-8 ISSN 2041-1723 Altmetric
2016	Disease mechanisms in ALS: misfolded SOD1 transferred through exosome-dependent and exosome-independent pathways Published In Cellular and Molecular Neurobiology Volume 36 Pages 377 - 381 ISSN 0272-4340 Altmetric
2016	Protein aggregates stimulate macropinocytosis facilitating their propagation Published In Prion Volume 10 Pages 119 - 126 ISSN 1933-6896 Altmetric
2016	Rapid flow cytometric measurement of protein inclusions and nuclear trafficking Published In Scientific Reports Volume 6 Pages 31138-1 - 31138-9 ISSN 2045-2322 Altmetric
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 Volume 10 Pages 499-1 - 499-16 ISSN 1662-453X Altmetric
2016	Walking the tightrope: proteostasis and neurodegenerative disease Published In Journal of Neurochemistry Volume 137 Pages 489 - 505 ISSN 0022-3042 Altmetric
2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published In Scientific Reports Volume 5 Pages 13416-1 - 13416-14 ISSN 2045-2322 Altmetric
2015	Evaluation of skin fibroblasts from amyotrophic lateral sclerosis patients for the rapid study of pathological features Published In Neurotoxicity Research Volume 28 Pages 138 - 146 ISSN 1029-8428 Altmetric
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 Volume 134 Pages 206 - 207 ISSN 0022-3042 Altmetric
2015	SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation Published In Molecular Neurodegeneration Volume 10 Pages 57-1 - 57-17 ISSN 1750-1326 Altmetric
2015	SerpinB2 (PAI-2) modulates proteostasis via binding misfolded proteins and promotion of cytoprotective inclusion formation Published In PLoS One Volume 10 Pages e0130136-1 - e0130136-20 ISSN 1932-6203 Altmetric
2015	The role of macropinocytosis in the propagation of protein aggregation associated with neurodegenerative diseases Published In Frontiers in Physiology Volume 6 Pages 277-1 - 277-8 ISSN 1664-042X Altmetric
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 Volume 15 Pages 170 - 171 Altmetric
2014	Amilial amyotrophic lateral sclerosis-associated proteins form aggregates via distinct pathways in cells Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration Volume 15 Pages 170 - 170 Altmetric
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 Volume 111 Pages 3620 - 3625 ISSN 1091-6490 Altmetric
2014	Misfolded polyglutamine, polyalanine, and superoxide dismutase 1 aggregate via distinct pathways in the cell Published In Journal of Biological Chemistry Volume 289 Pages 6669 - 6680 ISSN 0021-9258 Altmetric
2013	Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype Published In Glia Volume 61 Pages 409 - 419 ISSN 0894-1491 Altmetric
2013	Extracellular chaperones and proteostasis Published In Annual Review of Biochemistry Volume 82 Pages 295 - 322 ISSN 0066-4154 Altmetric
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 Volume 70 Pages 4181 - 4195 ISSN 1420-682X Altmetric
2013	Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis Published In Scientific Reports Volume 3 Pages 3275-1 - 3275-6 ISSN 2045-2322 Altmetric
2013	Intercellular propagated misfolding of wild-type Cu-Zn superoxide dismutase Published In Prion Volume 7 Pages 76 - 76 ISSN 1933-6896
2013	P2X7 receptor activation induces reactive oxygen species formation and cell death in murine EOC13 microglia Published In Mediators of Inflammation Volume 2013 Pages 271813-1 - 271813-18 ISSN 0962-9351 Altmetric
2013	Protease-activated alpha-2-macroglobulin can inhibit amyloid formation via two distinct mechanisms Published In FEBS Letters Volume 587 Pages 398 - 403 ISSN 0014-5793 Altmetric
2013	The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro Published In Cell Stress and Chaperones Volume 18 Pages 251 - 257 ISSN 1355-8145 Altmetric
2012	Roles of extracellular chaperones in amyloidosis Published In Journal of Molecular Biology Volume 421 Pages 499 - 516 ISSN 0022-2836 Altmetric
2011	Clusterin facilitates in vivo clearance of extracellular misfolded proteins Published In Cellular and Molecular Life Sciences Volume 68 Pages 3919 - 3931 ISSN 1420-682X Altmetric
2010	ANS binding reveals common features of cytotoxic amyloid species Published In ACS Chemical Biology Volume 5 Pages 735 - 740 ISSN 1554-8929 Altmetric
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 Volume 15 Pages 115 - 121 ISSN 1355-8145 Altmetric
2010	Protein chemistry of amyloid fibrils and chaperones: implications for amyloid formation and disease Published In Current Chemical Biology Volume 4 Pages 89 - 98 Altmetric
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 Volume 277 Pages 79 - 80 ISSN 1742-464X Altmetric
2009	Macroglobulin and haptoglobin suppress amyloid formation by interacting with prefibrillar protein species Published In Journal of Biological Chemistry Volume 284 Pages 4246 - 4254 ISSN 0021-9258 Altmetric
2009	Structural characterization of clusterin-chaperone client protein complexes Published In Journal of Biological Chemistry Volume 284 Pages 21920 - 21927 ISSN 0021-9258 Altmetric
2009	Therapeutic targets in extracellular protein deposition diseases Published In Current Medicinal Chemistry Volume 16 Pages 2855 - 2866 ISSN 0929-8673 Altmetric
2008	Clusterin interacts with paclitaxel and confer paclitaxel resistance in ovarian cancer Published In Neoplasia Volume 10 Pages 964 - 972 ISSN 1522-8002
2008	Protease activation of α2-Macroglobulin modulates a chaperone-like action with broad specificity Published In Biochemistry Volume 47 Pages 1176 - 1185 ISSN 0006-2960 Altmetric
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 Volume 4 Pages 42 - 52 ISSN 1742-2051 Altmetric
2007	The extracellular chaperone clusterin influences amyloid formation and toxicity by interacting with pre-fibrillar structures Published In The FASEB Journal Volume 21 Pages 2312 - 2322 ISSN 0892-6638 Altmetric
2007	The extracellular chaperone clusterin potently inhibits human lysozyme amyloid formation by interacting with prefibrillar species Published In Journal of Molecular Biology Volume 369 Pages 157 - 167 ISSN 0022-2836 Altmetric

Chapter

Year	Title
2016	Network approaches to the understanding of Alzheimer’s disease: from model organisms to humans Published In Systems Biology of Alzheimer's Disease Publisher Humana Press Pages 447 - 458 Altmetric
2015	The interplay of protein aggregates, microglia and neuroinflammation in neurodegenerative disease Published In Microglia: Physiology, Regulation and Health Implications Publisher Nova science publishers Pages 157 - 204
2013	Extracellular chaperones Published In Molecular Chaperones Publisher Berlin: Springer Berlin Heidelberg Pages 241 - 268 Altmetric
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 : Elsevier Pages 89 - 114 Altmetric
2008	Extracellular Chaperones and Amyloids Published In Heat Shock Proteins and the Brain: Implications for Neurodegenerative Diseases and Neuroprotection Publisher United States: Springer Science Pages 283 - 315 Altmetric

Investigator On

Year	Title
2019 - 2020	Is UBA1 a therapeutic target in ALS? 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
2019	GoBlot machines and Leica Objective Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2019	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
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
2017	Lumenera Infinity 2-5 microscope colour camera Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
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
2016	Contributions to Leica Dmi8 microscope Awarded by: National Health and Medical Research Council (NHMRC) Funding Scheme: Equipment Grant
2016	Does the transfer of ALS protein aggregates between motor neurons trigger neurodegeneration? Awarded by: Motor Neurone Disease Research Institute Funding Scheme: Grants-in-Aid
2016	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
2016	Ubiquitin depletion as a cause of Amyotrophic Lateral Schlerosis (ALS) 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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	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

Impact Story

Advisees

Graduate Advising Relationship

Degree	Research Title	Advisee
Doctor of Philosophy	Examination of the Ubiquitin Proteasome System in Induced Pluripotent Stem Cell Derived Motor Neurons, and Related Cell Types in the Context of Amyotrophic Lateral Sclerosis	Bax, Monique
Doctor of Philosophy	Investigating the role of proteostasis pathways in regulating the intracellular protein aggregation 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	Are proteostasis defects responsible for ALS.	Lambert-Smith, Isabella

Organizer Of Event

Proteostasis and Disease Symposium 2012 -

Orcid Id

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

Scopus Id

9739159000

Overview

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Research Overview

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Available for Collaborative Projects

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Investigator On

Impact

Impact Story

Supervision

Advisees

Graduate Advising Relationship

Service

Organizer Of Event

Identity

Orcid Id

Scopus Id

Overview

Top Publications

Research Areas

Research Overview

Affiliation

Member Of

Has Collaborator

Publications

Selected Publications

Journal Article

Chapter

Funding

Investigator On

Impact

Impact Story

Supervision

Advisees

Graduate Advising Relationship

Service

Organizer Of Event

Identity

Orcid Id

Scopus Id

Visualisations

Research Areas