Mitochondrial Iron Overload And Friedreich's Ataxia: The Role Of Frataxin In Iron And Haem Metabolism
Funder
National Health and Medical Research Council
Funding Amount
$285,990.00
Summary
Friedreich's ataxia (FA) is due to the lack of a protein known as frataxin. Recent studies using Baker's yeast have shown that the deletion of frataxin results in the accumulation of toxic iron in the mitochondrion. More recently, a variety of studies have shown that FA patients have iron loading within their cells. The iron build-up may cause severe damage. At present, the role of frataxin in mammalian mitochondrial iron metabolism is unknown. Our preliminary studies demonstrate that frataxin i ....Friedreich's ataxia (FA) is due to the lack of a protein known as frataxin. Recent studies using Baker's yeast have shown that the deletion of frataxin results in the accumulation of toxic iron in the mitochondrion. More recently, a variety of studies have shown that FA patients have iron loading within their cells. The iron build-up may cause severe damage. At present, the role of frataxin in mammalian mitochondrial iron metabolism is unknown. Our preliminary studies demonstrate that frataxin is down-regulated by either erythroid differentiation or the haem precursor protoporphyrin IX (Becker and Richardson, submitted). These data strongly suggest a role for frataxin in iron metabolism. In the present study we will continue to assess if frataxin plays a role in the way cells handle iron. Using a unique model of mitochondrial iron overload developed in my lab (Richardson et al. (1996) BLOOD 87:3477), we will extensively investigate the iron metabolism of the mitochondrion in order to determine the function of frataxin and its role in Friedreich's ataxia. In addition, we have developed a series of new drugs known as iron chelators that can enter the mitochondrion due to their high lipid solubility (Becker and Richardson 1999 J. Lab. Clin. Med. 134:510). These latter drugs are far more effective than the chelator currently used to treat iron overload, desferrioxamine (DFO). Indeed, our chelators have been designed to result in high iron chelation efficacy but low toxicity (see Becker and Richardson, 1999). This exciting research may be crucial in understanding the development of FA and in creating new therapies such as the use of iron chelators.Read moreRead less
Industrial Transformation Research Hubs - Grant ID: IH230100010
Funder
Australian Research Council
Funding Amount
$5,000,000.00
Summary
ARC Research Hub for Smart Process Design and Control . ARC Research Hub for Smart Process Design and Control aims to develop and apply advanced computational technologies to model and optimise complex multiphase processes by integrating the novel multiscale and AI modelling approaches. The outcomes include theories, computer models and simulation techniques, advanced knowledge about process modelling and optimisation, innovative technologies and processes for low carbon operations, and tens of ....ARC Research Hub for Smart Process Design and Control . ARC Research Hub for Smart Process Design and Control aims to develop and apply advanced computational technologies to model and optimise complex multiphase processes by integrating the novel multiscale and AI modelling approaches. The outcomes include theories, computer models and simulation techniques, advanced knowledge about process modelling and optimisation, innovative technologies and processes for low carbon operations, and tens of postdoc and PhD students through academic, industrial and international collaboration. Their application will significantly improve energy/process efficiency and reduce CO2 emission. The Hub will generate a significant impact on the mineral and metallurgical industries which are important to Australia.Read moreRead less
Industrial Transformation Training Centres - Grant ID: IC220100028
Funder
Australian Research Council
Funding Amount
$4,969,602.00
Summary
ARC Training Centre for Innovative Composites for the Future of Sustainable Mining Equipment. The Centre aims to train industry-focused researchers in advanced manufacturing of new-generation mining equipment and sustainable mining technology, through close collaborations among key universities and mining and manufacturing companies. The Centre will cultivate a team of world-class academic researchers and industry leaders to deliver an innovative program on research of innovative composites coup ....ARC Training Centre for Innovative Composites for the Future of Sustainable Mining Equipment. The Centre aims to train industry-focused researchers in advanced manufacturing of new-generation mining equipment and sustainable mining technology, through close collaborations among key universities and mining and manufacturing companies. The Centre will cultivate a team of world-class academic researchers and industry leaders to deliver an innovative program on research of innovative composites coupled with work-integrated learning, to not only produce a workforce that meets future skills demand but also develop sustainable and cost-effective mining equipment and high-efficiency mining technologies, benefiting the nation's manufacturing and mining sectors and significantly enhancing the competitiveness of the Australian mining industry.Read moreRead less
Coke integrity in blast furnace ironmaking: understanding and technology development. This project's expected outcomes will lead to optimised coke consumption for the blast furnace and to a more competitive steel manufacturing sector, through improved and rational criteria for selection of carbonaceous materials. This will result in more efficient ironmaking with significant operational, economical and environmental benefits.
The use of Australian magnetite ore in advanced ironmaking. The project will study reduction behaviour of Australian magnetite ore in advanced ironmaking with low emission of greenhouse and hazardous substances. Efficient utilisation of magnetite ore which economic demonstrated resources constitute 9.5 gigatonnes will be of high importance to Australian industry and prosperity.
Industrial Transformation Research Hubs - Grant ID: IH140100035
Funder
Australian Research Council
Funding Amount
$5,000,000.00
Summary
ARC Research Hub for Computational Particle Technology. ARC Research Hub for Computational Particle Technology. This research hub aims to develop and apply advanced theories and mathematical models to design and optimise particulate and multiphase processes that are widely used in the minerals and metallurgical industries. This should be achieved through detailed analysis of the fundamentals governing fluid flow, heat and mass transfer at different time and length scales, facilitated by various ....ARC Research Hub for Computational Particle Technology. ARC Research Hub for Computational Particle Technology. This research hub aims to develop and apply advanced theories and mathematical models to design and optimise particulate and multiphase processes that are widely used in the minerals and metallurgical industries. This should be achieved through detailed analysis of the fundamentals governing fluid flow, heat and mass transfer at different time and length scales, facilitated by various novel research techniques. Research outcomes including theories, computer models and simulation techniques, as well as well-trained young researchers, should have a significant impact across a range of industries of vital importance to Australia’s economic and technological future, including the minerals, metallurgical, materials, chemical, energy, pharmaceutical and environment sectors.Read moreRead less
Granular dynamics: theories, modelling and simulation. Particle science and technology is a rapidly developing interdisciplinary research field and is of paramount importance Australia in view of the heavy dependence on raw materials processing. This project will tackle the core problems in this field by developing novel theories and mathematical models to describe the flow of particles. Application of the research outcomes can lead to better process or product control, a decrease in energy cons ....Granular dynamics: theories, modelling and simulation. Particle science and technology is a rapidly developing interdisciplinary research field and is of paramount importance Australia in view of the heavy dependence on raw materials processing. This project will tackle the core problems in this field by developing novel theories and mathematical models to describe the flow of particles. Application of the research outcomes can lead to better process or product control, a decrease in energy consumption and an improvement in productivity, which, together with the research training offered through the conduct of the work, is very helpful to maintaining Australia's leading position in resource, energy, process and allied industries.Read moreRead less
Discrete particle modelling and analysis of complex particle-fluid flows. Multiphase processes are widely used in both conventional and modern industries in Australia and worldwide, however rarely reach more than 60% of design capacity because of a poor understanding of their fundamental characteristics. This project aims to overcome this problem using an extensive combined fundamental and applied approach. The resulting theories, computer models and simulation techniques will be applied to imp ....Discrete particle modelling and analysis of complex particle-fluid flows. Multiphase processes are widely used in both conventional and modern industries in Australia and worldwide, however rarely reach more than 60% of design capacity because of a poor understanding of their fundamental characteristics. This project aims to overcome this problem using an extensive combined fundamental and applied approach. The resulting theories, computer models and simulation techniques will be applied to improve process design, control and optimisation. Consequentially, productivity and Australian competitiveness will be significantly enhanced in its most important industries such as minerals, metallurgical, chemical, energy, and materials.Read moreRead less
Multiscale modelling and analysis of complex particulate and multiphase flow. Particulate and multiphase processes are widely used in both conventional and modern industries in Australia and worldwide, however rarely reach more than 60% of design capacity because of a poor understanding of their fundamental characteristics. This frontier technology research program aims to overcome this problem using an extensive combined fundamental and applied approach. The resulting theories, computer models ....Multiscale modelling and analysis of complex particulate and multiphase flow. Particulate and multiphase processes are widely used in both conventional and modern industries in Australia and worldwide, however rarely reach more than 60% of design capacity because of a poor understanding of their fundamental characteristics. This frontier technology research program aims to overcome this problem using an extensive combined fundamental and applied approach. The resulting theories, computer models and simulation techniques will be applied to improve process design, control and optimisation. Consequentially, productivity and Australian competitiveness will be significantly enhanced in its most important industries such as minerals, metallurgical, chemical, energy, pharmaceutical and materials.Read moreRead less
Thermal characterisation of iron ores and coals for HIsmelt operation. Substantial developments in direct reduction ironmaking (DRI) have been recently conducted providing sustainable way for metallurgical operations. The largest advantage of DRI is that it does not require cokemaking and sintering, two processes which are consistently causing environmental concerns. This project aims to thermally investigate coals and iron ores for optimising direct smelting, and to provide insight into effect ....Thermal characterisation of iron ores and coals for HIsmelt operation. Substantial developments in direct reduction ironmaking (DRI) have been recently conducted providing sustainable way for metallurgical operations. The largest advantage of DRI is that it does not require cokemaking and sintering, two processes which are consistently causing environmental concerns. This project aims to thermally investigate coals and iron ores for optimising direct smelting, and to provide insight into effect of the properties of ores and coals on the process. Structural changes using hot stage optical microscopy will be carefully clarified. Finally, a predictive model for DRI and energy requirements for direct smelting based on the experimental outcomes will be developed.Read moreRead less