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Article

Interleukin-1–mediated hyperinflammation in XIAP deficiency is associated with defective autophagy

31 May 2024

Abstract excerpt

ABSTRACT: Deficiency of X-linked inhibitor of apoptosis protein (XIAP) is a rare genetic condition that can present with recurrent episodes of hemophagocytic lymphohistiocytosis (HLH), though the exact mechanisms leading to this hyperinflammatory disorder are unclear. Understanding its biology is critical to developing targeted therapies for this potentially fatal disease. Here, we report on a novel multiexonic...

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PMID: 38820590
DOI: 10.1182/blood.2023023707

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