A5038771863- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Prion Diseases and Protein Misfolding
- Genetic Neurodegenerative Diseases
- Heterotopic Ossification and Related Conditions
- Medical Imaging and Pathology Studies
- Fungal and yeast genetics research
- RNA Research and Splicing
- Alzheimer's disease research and treatments
- Cholinesterase and Neurodegenerative Diseases
The University of Queensland
2019-2024
Park Centre for Mental Health
2023
Abstract Aggregation of the RNA-binding protein, TDP-43, is unifying hallmark amyotrophic lateral sclerosis and frontotemporal dementia. TDP-43-related neurodegeneration involves multiple changes to normal physiological which undergoes nuclear depletion, cytoplasmic mislocalisation, post-translational modification, aberrant liquid–liquid phase separation, preceding inclusion formation. Along with toxic aggregation, concurrent depletion dysfunction TDP-43 in cells pathology likely a key...
Abstract Understanding the mechanisms that drive TDP-43 pathology is integral to combating amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD) and other neurodegenerative diseases. Here we generated a longitudinal quantitative proteomic map of cortex from cytoplasmic rNLS8 mouse model ALS FTLD, developed complementary open-access webtool, TDP-map ( https://shiny.rcc.uq.edu.au/TDP-map/ ). We identified distinct protein subsets enriched for diverse biological pathways...
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease commonly treated with riluzole, small molecule that may act via modulation of glutamatergic neurotransmission. However, riluzole only modestly extends lifespan for people living ALS, and its precise mechanisms action remain unclear. Most ALS cases are characterised by accumulation cytoplasmic TAR DNA binding protein 43 kDa (TDP‐43), understanding the effects in models closely recapitulate TDP‐43 pathology provide...
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease commonly treated with riluzole, small molecule that may act via modulation of glutamatergic neurotransmission. However, riluzole only modestly extends lifespan for people living ALS and its precise mechanisms action remain unclear. Most cases are characterised by accumulation cytoplasmic TAR DNA binding protein 43 kDa (TDP-43), understanding the effects in models closely recapitulate TDP-43 pathology provide insights...
Abstract Understanding the mechanisms that drive TDP-43 pathology is integral to combating neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). To address this, we sought determine timeline of proteomic alterations across disease course in proteinopathy. Using longitudinal quantitative proteomics analysis cortex samples from cytoplasmic rNLS8 mouse model ALS FTLD, identified several distinct protein subsets characterized by...
Abstract Dysfunction and aggregation of the RNA-binding protein, TDP-43, is unifying hallmark amyotrophic lateral sclerosis (ALS) frontotemporal dementia (FTD). Mechanisms relative contributions concurrent TDP-43 nuclear depletion, cytoplasmic accumulation, post-translational modification to neurodegeneration remain unresolved. We employed CRISPR/Cas9-mediated fluorescent tagging investigate how disease-associated stressors pathological alter abundance, localisation, self-assembly,...