Metabolic dysfunction in iPSC-derived models of Motor Neuron Disease

Motor Neuron Disease (MND) is a fatal neurological condition in which the average life expectancy is only 27 months after diagnosis. The progressive degeneration of neurons in the central nervous system leads to paralysis and eventually, death. Understanding what causes these neurons to degenerate, therefore, is essential. Evidence has come to light suggesting that a patient's metabolism, particularly their ability to use glucose, is significantly impaired throughout disease progression. What is not completely understood, however, is how these metabolic deficits may present themselves in individual tissues. Timothy's research, focuses on studying MND affected neurons - particularly iPSC-derived cortical neurons, in an effort to understand how MND affects these neurons metabolically, and whether this may lead to neuronal degeneration. Using the knowledge gained from these studies, I aim to undertake pre-clinical testing of a number of potential therapeutic compounds.

​Graduated from the University of Queensland in 2015 with a Bachelor of Science, majoring in Biomedical Science (Honours I). Honours was undertaken with A/Prof Peter Noakes and Prof Ernst Wolvetang, working towards developing an in vitro model of the human neuromuscular junction. In 2016, a PhD was undertaken with Dr Shyuan Ngo and Prof Ernst Wolvetang, studying the metabolic deficits observed in motor neuron disease affected iPSC-derived cortical neurons.

Industry Engagement and Collaborations

​Timothy's PhD project is a collaboration between the Wolvetang group at AIBN, and the Ngo group. He also collaborates with the Laboratory of Mitochondrial Biology and Metabolism at the NIH in America, working on characterising the neuronal and metabolic characteristics of a novel disease model