Fanconi anemia (FA) is a rare inherited disorder characterized by progressive bone marrow failure (BMF) and a predisposition to malignancy. Systemic reactive-oxygen species (ROS) and increased sensitivity of FA hematopoietic progenitors to ROS play a key role in the pathogenesis of BMF. Treatment with antioxidants improve hematopoietic function in Fancc-/- mice.
View Article and Find Full Text PDFIn this study we examined the activation of the non-canonical NFκB signalling pathway in endothelial cells. In HUVECs, LIGHT stimulated a delayed induction of serine 866/870 p100 phosphorylation linked to p52 NFκB formation. Surprisingly, the canonical ligand, IL-1β, stimulated a rapid phosphorylation or p100 which was not associated with p52 formation.
View Article and Find Full Text PDFBackground: Healthy skin is important for maintaining overall physical and cultural health and wellbeing. However, remote-living Australian Aboriginal children contend with disproportionally high rates of (Strep A) infected impetigo. The SToP Trial was a large stepped-wedge cluster randomised trial of See, Treat, and Prevent (SToP) skin health activities implemented between 2019 and 2022 in the Kimberley region of Western Australia, during which a decrease in impetigo was observed.
View Article and Find Full Text PDFThe inhibitory-kappaB kinases (IKKs) IKKα and IKKβ play central roles in regulating the non-canonical and canonical NF-κB signalling pathways. Whilst the proteins that transduce the signals of each pathway have been extensively characterised, the clear dissection of the functional roles of IKKα-mediated non-canonical NF-κB signalling versus IKKβ-driven canonical signalling remains to be fully elucidated. Progress has relied upon complementary molecular and pharmacological tools; however, the lack of highly potent and selective IKKα inhibitors has limited advances.
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