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This report establishes a novel form of IEI due to DN RELA mutations as a cause of chronic mucocutaneous ulcerations with autoinflammatory and/or autoimmune manifestations. This also expands the clinical spectrum of human RELA-associated diseases.

Xu et al. define Baf60c in skeletal myofibers as a critical regulator of muscle regeneration through Dkk3-mediated paracrine signaling on muscle stem cells. They also uncover that Baf60c interacts with transcription factor Six4 to synergistically suppress Dkk3 expression in myocytes.

Steady-state and emergency myelopoiesis require different rates of mature cell production to maintain or restore homeostasis. This study demonstrates that mitochondrial pyruvate is specifically required for steady-state but not emergency myelopoiesis. Reciprocally, glutaminolysis specifically promotes emergency but not steady-state myelopoiesis.

Neutrophil extracellular traps and extracellular histones potentiate Th17 inflammation and bone destruction in animal models of periodontitis. Findings in patients with periodontitis provide human relevance and support further exploration of NET-mediated pathology in human disease.

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