Area of research
Neurology · Genetics
Research interest
Research focused on Amyotrophic lateral sclerosis and SOD1, with related work in Proteostasis, Ubiquitin, Cell biology. Notable publications include 'CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia', 'Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions', and 'Proteome Homeostasis Dysfunction: A Unifying Principle in ALS Pathogenesis'.
Development of a targeted BioPROTAC degrader selective for misfolded SOD1
A polytherapy approach demonstrates therapeutic efficacy for the treatment of SOD1 associated amyotrophic lateral sclerosis
ALS-linked CCNF variant disrupts motor neuron ubiquitin homeostasis
TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex
Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateral sclerosis?
Cells Overexpressing ALS-Associated SOD1 Variants Are Differentially Susceptible to CuATSM-Associated Toxicity
p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death
CuATSM improves motor function and extends survival but is not tolerated at a high dose in SOD1G93A mice with a C57BL/6 background
Mutant Cu/Zn Superoxide Dismutase (A4V) Turnover Is Altered in Cells Containing Inclusions
Proteome Homeostasis Dysfunction: A Unifying Principle in ALS Pathogenesis
Ubiquitin Homeostasis Is Disrupted in TDP-43 and FUS Cell Models of ALS
The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation
SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS
Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells
CuATSM Protects Against the <i>In Vitro</i> Cytotoxicity of Wild-Type-Like Copper–Zinc Superoxide Dismutase Mutants but not Mutants That Disrupt Metal Binding
Flow cytometric measurement of the cellular propagation of TDP-43 aggregation
Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation
CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions
SerpinB2 (PAI-2) Modulates Proteostasis via Binding Misfolded Proteins and Promotion of Cytoprotective Inclusion Formation
Alpha-2-Macroglobulin Is Acutely Sensitive to Freezing and Lyophilization: Implications for Structural and Functional Studies
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