Uncovering The Molecular Mechanisms Behind Charcot-Marie-Tooth Disease
Funder
National Health and Medical Research Council
Funding Amount
$320,967.00
Summary
Charcot-Marie-Tooth disease (or CMT) is one of the most common disorders of the nervous system, affecting the normal function of the limbs and causing lifelong disabilities. There is currently no cure for CMT. The aim of this research is to develop a new model of CMT, which will allow us to uncover novel information about how the disease develops. This research will provide a better understanding of the disease and therefore provide valuable insight for the future generation of therapeutics.
Understanding The Link Between Mitochondrial Biogenesis And Disease
Funder
National Health and Medical Research Council
Funding Amount
$421,055.00
Summary
As the predominate energy producers of our cells, mitochondria are implicated in a variety of diseases. To function properly, these dynamic organelles rely on protein components that regulate their structure and distribution throughout the cell. My work aims to expand our knowledge of the way these components control mitochondrial shape and trafficking. By understanding the correlation between mitochondrial morphology and function, we will gain insight into related diseases.
Biogenesis Of Respiratory Chain Complex I And Analysis Of Assembly Defects In Patients With Mitochondrial Disease
Funder
National Health and Medical Research Council
Funding Amount
$254,250.00
Summary
Complex I of the mitochondrial respiratory chain is a large assembly of protein subunits that is involved in the main production of cellular energy. Complex I is found in intracellular compartments termed mitochondria. The predicament for Complex I is that in order for it to be built, it requires 38 different proteins that are made in one place in the cell to be imported into mitochondria and then somehow joined together with the 7 other subunits that are made by mitochondria. This is clearly a ....Complex I of the mitochondrial respiratory chain is a large assembly of protein subunits that is involved in the main production of cellular energy. Complex I is found in intracellular compartments termed mitochondria. The predicament for Complex I is that in order for it to be built, it requires 38 different proteins that are made in one place in the cell to be imported into mitochondria and then somehow joined together with the 7 other subunits that are made by mitochondria. This is clearly a complicated procedure and we have little information on how its assembly is achieved. We do know however that mistakes in the assembly of Complex I do happen. In Australia, about 50 children born each year have inherited disorders of mitochondrial energy generation. The most severe disorders cause infant death, while others present later causing a range of degenerative diseases, particularly affecting brain, muscle and heart. The most common defect in these patients is a loss in the activity of Complex I. Interestingly it seems that in most cases, the defect is not due to a mutation in one or more of the Complex I subunits and so we believe that such defects arise form accessory proteins that are involved in the construction of Complex I. The aim of this proposal is to investigate how Complex I is assembled and to identify and characterise accessory proteins. We will also analyse assembly defects by studying skin fibroblasts from patients. This work will aid in our understanding of not only how protein complexes are built, but how defects in their assembly can cause disease. This will not only be informative to families of affected individuals but may aid in future diagnosis and prevention of mitochondrial disease.Read moreRead less
Molecular Energies and Non-Bonded Interactions. The development of new techniques that allow non-bonded chemical interactions to be modelled and predicted reliably and accurately will allow researchers in the chemical, and pharmaceutical sciences to predict the physical and chemical behaviour of moderately large molecular systems with an accuracy and efficiency that has not previously been possible. The software that will result will enable cost and time savings in molecular design within the m ....Molecular Energies and Non-Bonded Interactions. The development of new techniques that allow non-bonded chemical interactions to be modelled and predicted reliably and accurately will allow researchers in the chemical, and pharmaceutical sciences to predict the physical and chemical behaviour of moderately large molecular systems with an accuracy and efficiency that has not previously been possible. The software that will result will enable cost and time savings in molecular design within the medical and agricultural contexts.Read moreRead less
Fission, Fusion And Distribution Of Mitochondria In Mammalian Cells
Funder
National Health and Medical Research Council
Funding Amount
$480,750.00
Summary
Mitochondria are small cellular compartments that produce most of the cell's energy in the form of ATP. Mitochondria were once thought of as small bean-shaped organelles floating around in the cell, however it has become clear that this is not the case. Mitochondria are found as a network of tubules radiating from around the nucleus and they undergo constant changes in their shape through both fission and fusion events. Mitochondria are transported along microtubules which act as highways in the ....Mitochondria are small cellular compartments that produce most of the cell's energy in the form of ATP. Mitochondria were once thought of as small bean-shaped organelles floating around in the cell, however it has become clear that this is not the case. Mitochondria are found as a network of tubules radiating from around the nucleus and they undergo constant changes in their shape through both fission and fusion events. Mitochondria are transported along microtubules which act as highways in the cell so that they can be distributed to areas that require ATP or other functions particular to mitochondria such as their ability to regulate the release of calcium. In specialist cells, mitochondria are organised even further. Sperm cells contain mitochondria packed around the mid-piece of the flagellum so that ATP can be utilised directly for swimming. Mitochondria are also highly organised in muscle cells to supply ATP for movement while in pancreatic cells mitochondria at the cell's edge help to regulate the secretion of molecules such as insulin into the bloodstream. While we are beginning to understand the great importance of mitochondria to the cell, we are yet to work out how these organelles undergo the drastic morphological changes which are essential for cellular function. In this application, we plan to identify and characterise the proteins involved in the division of mitochodria and the movement of these organelles along the microtubule highways. Understanding the basic mechanisms of mitochondrial dynamics in tissue culture cells will provide valuable insights into mitochondrial segregation and specialisation in differentiated cells such as sperm, nerve, muscle and pancreatic cells, where such events are crucial for function.Read moreRead less
Stock Identification And Discrimination Of Mulloway In Australian Waters
Funder
Fisheries Research and Development Corporation
Summary
Objectives: 1. Investigate population structure of mulloway, Argyrosomus hololepidotus, to determine whether mulloway in Australian waters belong to one large inter-breeding population throughout their range or whether 2 or more separate stocks exist.
Shark Futures: A Report Card For Australia's Sharks And Rays
Funder
Fisheries Research and Development Corporation
Funding Amount
$199,997.20
Summary
With growing concerns for the status of shark and ray populations world-wide, and increasing pressure to ensure Australia’s 320 species are effectively managed and conserved, there is a need for decision makers in government to have access to comprehensive and accurate information. One of the greatest challenges for the sharks and rays is that more than any other taxa they exist across the spectrum of interests from sustainable fisheries resources to threatened species requiring conservation. Fu ....With growing concerns for the status of shark and ray populations world-wide, and increasing pressure to ensure Australia’s 320 species are effectively managed and conserved, there is a need for decision makers in government to have access to comprehensive and accurate information. One of the greatest challenges for the sharks and rays is that more than any other taxa they exist across the spectrum of interests from sustainable fisheries resources to threatened species requiring conservation. Further complicating the assessment and management of these species is the fact that many species ranges extend beyond Australia’s territorial waters, where management is implemented differently and populations may be in very different states. Australia’s abilities both in management of its sharks and rays, and the science that underpins it, are recognised as world-leading. Despite this there remain many challenges that face our sharks and rays, but they may not always be those that are faced by other nations in our region. Currently the available information is fragmentary and difficult to access, and most assessment is focused on only a few species targeted by fisheries. The growing information needs of initiatives such as Shark-Plan 2, CITES, CMS, ESD, WTO and EPBC listing struggle to be met because of the lack of a synthesis of information across this group. Locally relevant information on the status of sharks and rays, and the synthesis of knowledge about them, will thus be critical to addressing the challenges that face this group in Australian waters. Objectives: 1. To synthesise available information on sharks and rays in Australian waters 2. To produce a report card on the status of Australia’s sharks and rays Read moreRead less