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EVC-EVC2 complex stability and ciliary targeting are regulated by modification with ubiquitin and SUMOEllis van Creveld syndrome and Weyers acrofacial dysostosis are two rare genetic diseases affecting skeletal development. They are both ciliopathies, as they are due to malfunction of primary cilia, microtubule-based plasma membrane protrusions that function as cellular antennae and are required for Hedgehog signaling, a key pathway during skeletal morphogenesis.
Research
Oscillometry and spirometry are not interchangeable when assessing the bronchodilator response in children and young adults born pretermThe European Respiratory Society Oscillometry Taskforce identified that clinical correlates of bronchodilator responses are needed to advance oscillometry in clinical practice. The understanding of bronchodilator-induced oscillometry changes in preterm lung disease is poor. Here we describe a comparison of bronchodilator assessments performed using oscillometry and spirometry in a population born very preterm and explore the relationship between bronchodilator-induced changes in respiratory function and clinical outcomes.
Research
Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway diseaseMacrophages are the major resident immune cells in human airways coordinating responses to infection and injury. In cystic fibrosis, neutrophils are recruited to the airways shortly after birth, and actively exocytose damaging enzymes prior to chronic infection, suggesting a potential defect in macrophage immunomodulatory function.
Research
Bacteriophage: A new therapeutic player to combat neutrophilic inflammation in chronic airway diseasesPersistent respiratory bacterial infections are a clinical burden in several chronic inflammatory airway diseases and are often associated with neutrophil infiltration into the lungs. Following recruitment, dysregulated neutrophil effector functions such as increased granule release and formation of neutrophil extracellular traps (NETs) result in damage to airway tissue, contributing to the progression of lung disease.
Research
Current understanding of the neutrophil transcriptome in health and diseaseNeutrophils are key cells of the innate immune system. It is now understood that this leukocyte population is diverse in both the basal composition and functional plasticity. Underlying this plasticity is a post-translational framework for rapidly achieving early activation states, but also a transcriptional capacity that is becoming increasingly recognized by immunologists.
News & Events
Pre-term kids get green light to exerciseParents of children born prematurely have expressed concerns about their child’s lung health when they exercise, with symptoms such as breathlessness.
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New funding to accelerate AI personalised phage therapiesWal-yan researchers have been awarded $500,000 for their innovative research, supported by the Western Australian Government’s Future Health Medical Research and Innovation (FHRI) Fund.
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Bushfires: Tips to protect family from smoke exposurePerth’s north-east is under threat from an out-of-control bushfire.
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Toxic, harmful chemicals found in popular Australian e-liquidsPerth researchers have found toxic and harmful chemicals in several dozen e-cigarette liquids readily available in Australia.
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Enhancing the lung health for preterm birth survivors by uncovering treatable traitsA project to uncover treatable traits to improve the lung health of people born preterm has been made possible thanks to a $1.99 million Medical Research Future Fund (MRFF) grant.