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Pathobiologic Mechanisms of Neurodegeneration in Osteopetrosis Derived From Structural and Functional Analysis of 14 ClC-7 MutantsClC-7 is a chloride-proton antiporter of the CLC protein family. In complex with its accessory protein Ostm-1, ClC-7 localizes to lysosomes and to the osteoclasts' ruffled border, where it plays a critical role in acidifying the resorption lacuna during bone resorption. Gene inactivation in mice causes severe osteopetrosis, neurodegeneration, and lysosomal storage disease. Mutations in the human CLCN7 gene are associated with diverse forms of osteopetrosis.
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A Survey of Infectious Diseases and Microbiology Clinicians in Australia and New Zealand About the Management of Staphylococcus aureus BacteremiaSignificant variation in practice, particularly for patients with a severe disease phenotype and antibiotic-resistant profile
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Disparities between Aboriginal and non-Aboriginal perinatal mortality rates in Western Australia from 1980 to 2015This study aimed to examine the pattern of stillbirth and neonatal mortality rate disparities over time in Western Australia
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Disability "In-Justice": Benefits and Challenges of "Yarning" With Young People Undergoing Diagnostic Assessment for Fetal Alcohol Spectrum Disorder in Youth DetentionWe report the findings from a qualitative study that took place alongside a fetal alcohol spectrum disorder (FASD) prevalence study among detainees in Australia
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The dystroglycan receptor maintains glioma stem cells in the vascular nicheThese findings reveal a central role of the DG receptor, not only as a structural element, but also as a critical factor promoting mesenchymal-like GBM
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A Comparison of Pneumococcal Nasopharyngeal Carriage in Very Young Fijian Infants Born by Vaginal or Cesarean DeliveryPneumococcal nasopharyngeal carriage prevalence and density were higher in infants delivered vaginally compared with those delivered by cesarean birth
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Oxidative stress and abnormal bioactive lipids in early cystic fibrosis lung diseaseSeveral lipid biomarkers of early cystic fibrosis lung disease were identified, which point toward potential disease monitoring and therapeutic approaches
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Accumulation of CD103+ CD8+ T cells in a cutaneous melanoma micrometastasisResults support the emerging concept that CD103+ CD8+ tissue‐resident memory T cells are key mediators of cancer surveillance
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The gut microbiome and cardiovascular disease: current knowledge and clinical potentialThis review will focus on the role of the gut microbiome and associated functional components in the development and progression of atherosclerosis
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Boosting the influenza vaccine schedule in children with cancer: a prospective open-label studyCurrent immunization guidelines recommend one dose of influenza vaccine for children aged ≥9 years and two doses for younger or vaccine-naïve children. However, children receiving chemotherapy have an attenuated immune response. We performed a prospective open-label study in children undergoing treatment for cancer at Perth Children's Hospital, Western Australia, to examine the safety and efficacy of a boosted influenza schedule.