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Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse ModelSeverity and disease progression in people with Cystic Fibrosis is typically dependent on their genotype. One potential therapeutic strategy for people with specific mutations is exon skipping with antisense oligonucleotides. CFTR exon 9 is an in-frame exon and hence the exclusion of this exon would excise only 31 amino acids but not alter the reading frame of the remaining mRNA.
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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cellsThis study assessed an alternative approach, using a small scale halide assay that can be adapted for a personalized high throughput setting to analyze CFTR function of pAEC.
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Current options in aerosolised drug therapy for children receiving respiratory supportIn this review, we explore current knowledge and provide guidance as to when and how the inhaled route may be of value when treating patients whose tracheas are intubated
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Visualisation of Multiple Tight Junctional Complexes in Human Airway Epithelial CellsApically located tight junctions in airway epithelium perform a fundamental role in controlling macromolecule migration through paracellular spaces.
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Rhinovirus Infection Is Associated With Airway Epithelial Cell Necrosis and Inflammation via Interleukin-1 in Young Children With Cystic FibrosisThis study found rhinovirus infection drives necrotic cell death in cystic fibrosis airway epithelial cells
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Lung study helps history-making generation get a handle on their healthA lung function study carried out by Dr Shannon Simpson provided the most comprehensive follow-up of very pre-term children of any study so far carried out on the lung health of this vulnerable group.
Research
Compound Repurposing Into Novel Therapeutics In COVID-19 At risk Lungs (CRITICAL Study)Anthony Christopher David Ingrid Shannon Thomas Kicic Blyth Martino Laing Simpson Iosifidis BSc (Hons) PhD MBBS (Hons) DCH FRACP FRCPA PhD BSc PhD
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Epithelial Drivers of Neutrophil Plasticity in Early Cystic Fibrosis Lung DiseaseHallmarks of cystic fibrosis (CF) airway disease include bronchiectasis, airway inflammation by infiltrating polymorphonuclear neutrophils (PMNs) and recurring infection.
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WA Epithelial Research Program for Childhood Respiratory DiseasesOnce thought to be a simple barrier to the external environment, epithelial cells are involved in many repair and inflammatory processes that occur in childhood airway diseases.
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Children’s regenerative and genetic medicine programThe project aims to build capacity in regenerative medicine for children with respiratory diseases.