Professor Aleksandra Filipovska, Laboratory Head, Mitochondrial Medicine and Biology, Harry Perkins Institute of Medical Research, Perth, WA

Title: "The role of mitochondrial dysfunction in neurodegenerative diseases"


The size and organization of the animal mitochondrial genome has been reduced and compacted significantly since its endosymbiosis from an α-proteobacterial ancestor. This compaction has necessitated the evolution of unique mechanisms to facilitate rapid changes in gene expression in response to the changing energy demands of the cell. The mitochondrial transcriptome encodes proteins that are subunits of the respiratory chain, responsible for most of the energy production required by cells. Consequently the coordinated regulation of the mitochondrial transcriptome by the nucleus is of particular importance for the maintenance of cell health and energy metabolism, particularly in high energy demand organs such as the brain and heart. We have been investigating the unusual features of mitochondrial RNAs and the RNA-binding proteins that control their production, maturation, translation and stabilization to understand the regulation of mitochondrial gene expression and its contribution to health and disease. I will highlight the devastating consequences of dysregulated mitochondrial gene expression in different models of disease in mice and humans caused by genetic disruption of RNA-binding proteins. Mouse models of disease have enabled us to understand the in vivo role of fundamental processes that regulate mitochondrial RNA metabolism and the pathogenesis of diseases caused by impaired gene expression that cause neurodegeneration.




About Neuroscience Seminars

Neuroscience seminars at the QBI play a major role in the advancement of neuroscience in the Asia-Pacific region. The primary goal of these seminars is to promote excellence in neuroscience through the exchange of ideas, establishing new collaborations and augmenting partnerships already in place.

Seminars in the QBI Auditorium on Level 7 are held on Wednesdays at 12-1pm, which are sometimes simulcast on Zoom (with approval from the speaker). We also occassionally hold seminars from international speakers via Zoom. The days and times of these seminars will vary depending on the time zone of the speaker. Please see each seminar listed below for details. 


Neuroscience Seminars archive 2005-2018