Journal article
Reduced PU.1 expression underlies aberrant neutrophil maturation and function in b-Thalassemia mice and patients
P Siwaponanan, JY Siegers, R Ghazali, T Ng, B Mccoll, GZW Ng, P Sutton, N Wang, I Ooi, C Thiengtavor, S Fucharoen, P Chaichompoo, S Svasti, O Wijburg, J Vadolas
Blood | AMER SOC HEMATOLOGY | Published : 2017
Abstract
β-Thalassemia is associated with several abnormalities of the innate immune system. Neutrophils in particular are defective, predisposing patients to life-Threatening bacterial infections. The molecular and cellular mechanisms involved in impaired neutrophil function remain incompletely defined. We used the Hbbth3/+ β-Thalassemia mouse and hemoglobin E (HbE)/β-Thalassemia patients to investigate dysregulated neutrophil activity. Mature neutrophils from Hbbth3/1 mice displayed a significant reduction in chemotaxis, opsonophagocytosis, and production of reactive oxygen species, closely mimicking the defective immune functions observed in β-Thalassemia patients. In Hbbth3/1 mice, the expression..
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Awarded by Thailand Research Fund
Funding Acknowledgements
The authors acknowledge the financial support from the National Health and Medical Research Council, the Murdoch Childrens Research Institute, the Victorian Government's Operational Infrastructure Support Program, Thalassaemia and Sickle Cell Society of Australia, Thalassaemia Society of New South Wales, and The Greek Conference. This work was also supported by Thailand Research Fund (DPG5980001, MRG5980043, and IRG5780009), Office of the Higher Education Commission and Mahidol University under the National Research University Initiative, Research Chair Grant, National Science and Technology Development Agency, Thailand and Faculty of Science, and Faculty of Medicine Ramathibodi Hospital. Supported was provided by the Royal Golden Jubilee PhD Research Scholarship from the Thailand Research Fund (P. Siwaponanan).