Featured researchers

Dr. Patricia Agudelo-Romero is a mid-career postdoctoral. She is currently leading the bioinformatics research in the airway epithelial cell team within the Centre for Kids Respiratory Health, Telethon Kids Institute, Perth. 

She holds a PhD in Biotechnology from the Polytechnic University of Valencia (Spain) and graduated with the prestigious Extraordinary Doctorate Award in 2010. She also obtained a Master’s degree on Bioinformatics and Computational Biology in 2015 in Spain. During her postdoctoral stays, she worked in a European project between the University of Lisbon (Portugal) and University of Barcelona (Spain) for carrying out an innovative systems biology study through the integration of omics data (transcriptomics & metabolomics) to understand host response to fungal infections among other projects. For this pivotal work, she was awarded the CNOIV Innovation Award in 2016 (Portugal). Additionally, she held a Research Associate position at the University of Western Australia (UWA). During this time, she was working in variant calling in whole genome sequencing, gene expression analysis, de novo assembly for the detection of mobile elements and enrichment of known and de novo cis-regulatory elements.

Currently, her research is focused on exploring two key areas in Cystic fibrosis (CF) disease: (i) Host-pathogen interaction (i.e. mycobiome, bacteriome & virome), and (ii) The integration of multi-omics such as transcriptomics, metabolomics and epigenomics, as well as network analysis.

Under this framework, she is interested in the CF lung virome and its interaction with other microbiome parts such as bacteria and fungi. In addition, through the use of whole-transcriptome shotgun deep sequencing, she is looking for the first time make a catalogue of the full-length viral genomes present in the lung of children with CF. Since studying the viral diversity and its influence in the microbiome balance is important for the understanding of its impact during the lung function deterioration in CF.

On the other hand, she is also studying the transcriptomic and metabolic responses of primary epithelial cells of children with and without CF under a rhinovirus infection. By integrating those two omics, she is looking for specific biomarkers related to viral infections for therapeutic treatments.

Dr Shivanthan Shanthikumar and Professor Ranganathan are the joint recipients of the 'Vertex Innovation Award' 2018 for the project titled ‘do epigenetic changes predict CF lung disease severity?’
 

The aim of Dr Shanthikumar's research is to identify epigenetic ( the study of biological mechanisms that will switch genes on and off) markers from early life which predict the severity of lung disease at 9 years of age. 

Dr Luke Garratt is a National Health and Medical Research Council Early Career Fellow. Here he tells us a bit about this great opportunity.

"My NHMRC Early Career Fellowship is to lead an international collaborative study with CF researchers at Emory University, USA that closely investigates the behaviour of the immune system in the lungs of babies with CF. We seek to understand why the neutrophil, a key immune cell for controlling infections, so quickly becomes more harmful than helpful to the CF lung. If we can better control neutrophils, we can reduce the amount of progressive lung damage. I am monitoring how lung conditions vary between babies with CF in the initial stages of disease and how neutrophils respond to these conditions. More importantly, we hope to establish this approach as a way to test treatments targeting neutrophils in early CF."

Professor Phil Sutton. 
Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne.

Is Protease-Activated Receptor-1 (PAR1) important in cystic fibrosis pathogenesis?

When an infection or inflammation occurs in the body lots of things happen, including the release of enzymes called proteases. Proteases are molecules that cut up other proteins, and this obviously can result in a lot of damage. A family of receptors called Protease-Activated Receptors (PAR) are located on the surfaces of cells to detect such extracellular proteases. This tells the body there is an infection or inflammation, allowing it to mount a response. One member of this family, PAR1, can detect important proteases produced in the CF lung. Activation of PAR1 has been shown to have potent effects on inflammation in many infections, but nothing is known about its effect in CF. In work funded by grants from 65km for Cystic Fibrosis, we examined whether PAR1 could be important in CF pathogenesis. For this, we generated knock-out mice that lacked both the Cftr and Par1 genes. We also examined if activating PAR1 would change the inflammatory response of lung immune cells from children with CF to the bacterial pathogen Pseudomonas aeruginosa. These studies, recently submitted for publication, suggest PAR1 plays an important but very complex role in CF, with PAR1 activation being protective in some situations, while potentially harmful in others. More work is required to fully understand exactly what PAR1 is doing in CF.