HARNESSING THE CONSEQUENCES OF IMPAIRED MITOCHONDRIAL FUNCTION TO TREAT AND IMAGE MOTOR NEURON DISEASE

Grant number: 1084927 | Funding period: 2015 - 2018

Completed

Abstract

Motor neuron disease (MND) is a group of fatal adult-onset diseases affecting the neurons that relay signals from the brain to muscles. The incidence of MND is rapidly increasing due to the ageing population and a diagnosis of MND comes with a prognosis of paralysis and 2-5 years survival. The diagnosis process is lengthy, and even after MND is confirmed, there are no effective therapeutics. This research project aims to develop new therapeutic and diagnostic options for MND.

Related publications (5)

Scholarly works icon

An integrated mass spectrometry imaging and digital pathology workflow for objective detection of colorectal tumours by unique atomic signatures

Bence Paul, Kai Kysenius, James B Hilton, Michael WM Jones, Robert W Hutchinson, Daniel D Buchanan, Christophe Rosty, Fred Fryer, Ashley Bush, Janet M Hergt, Jon D Woodhead, David P Bishop, Philip A Doble, Michelle M Hill, Peter J Crouch, Dominic J Hare

2021-06-29

Tumours are abnormal growths of cells that reproduce by redirecting essential nutrients and resources from surrounding tissue. Cha..

Scholarly works icon

Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1

K Kysenius, JB Hilton, B Paul, DJ Hare, PJ Crouch

2019-01-01

Mutations in the copper (Cu)- and zinc (Zn)-binding metalloenzyme Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotr..

Scholarly works icon

A versatile quantitative microdroplet elemental imaging method optimised for integration in biochemical workflows for low-volume samples

Kai Kysenius, Bence Paul, James B Hilton, Jeffrey R Liddell, Dominic J Hare, Peter J Crouch

2019-01-01

Laser ablation-inductively coupled plasma-mass spectrometry (LA-ICP-MS) analysis of μ-droplets is becoming an attractive alternati..

Scholarly works icon

Cu-II(atsm) improves the neurological phenotype and survival of SOD1(G93A) mice and selectively increases enzymatically active SOD1 in the spinal cord

James B Hilton, Stephen W Mercer, Nastasia KH Lim, Noel G Faux, Gojko Buncic, Joseph S Beckman, Blaine R Roberts, Paul S Donnelly, Anthony R White, Peter J Crouch

2017-02-13

Ubiquitous expression of mutant Cu/Zn-superoxide dismutase (SOD1) selectively affects motor neurons in the central nervous system ..

Scholarly works icon

Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS

JB Hilton, AR White, PJ Crouch

2016-01-01

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease, a fatal degenerative disorder in which motor ..

University of Melbourne Researchers