Publications by authors named "A J Standing"

Article Synopsis
  • The enamel knot signaling center plays a crucial role in tooth shape development in mammals, influencing the growth of dental cusps through Fgf signaling.
  • Research shows that this signaling center is conserved in sharks, highlighting differences in tooth development between sharks and mice while indicating that Wnt signaling impacts tooth size and shape in similar ways across species.
  • The study suggests that despite variations in signaling pathways, there is a fundamental developmental framework shared among vertebrates, indicating that the enamel knot-like center has ancient origins predating modern teeth.
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Excessive exercise with limited recovery may lead to detrimental states of overreaching or the overtraining syndrome. Chronic maladaptation in endocrine and immune mechanisms occur with the incidence of these states. Exercise-induced cortisol and testosterone responses have been proposed as biomarkers of overreaching, with blunted responses following intensified-training periods.

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Article Synopsis
  • Nonclassical models, including the shark, are becoming essential for research in evolutionary, developmental, and regenerative biology.
  • The embryonic shark (Scyliorhinus canicula) allows for the study of tooth development and regeneration, which is less understood compared to the classic murine model.
  • Sharks showcase a unique polyphyodont dentition pattern, providing insights into continuous whole tooth regeneration and the evolutionary implications of this process in vertebrates.
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We identified a consanguineous kindred, of three affected children with severe autoinflammation, resulting in the death of one sibling and allogeneic stem cell transplantation in the other two. All three were homozygous for p.S208C mutation; however, their phenotype was more severe than previously reported, prompting consideration of an oligogenic autoinflammation model.

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Objectives: Monogenic autoinflammatory disorders (AID) and primary immunodeficiencies can present early in life with features that may be mistaken for Behçet's disease (BD). We aimed to retrospectively describe the clinical and laboratory features of 11 paediatric cases referred for suspected BD who turned out to have an alternative, monogenic disease mimicking BD.

Methods: Retrospective, paediatric BD specialist multicentre case series.

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