Abdelrahim Zoued

Inflammatory cell death and monocyte dysfunction in VEXAS syndrome

Paul Breillat, Samuel J. Magaziner, Stéphane M. Camus, Léa Dionet et autres

ABSTRACT: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a severe adult-onset autoinflammatory disease caused by somatic mutations in the UBA1 gene, disrupting cytoplasmic ubiquitin-activating enzyme E1 function in hematopoietic progenitors. Its pathogenesis remains poorly understood, particularly the mechanisms by which …

fr, us (code pays fourni par la source)

Rôle de la mort cellulaire médiée par RIPK1 et défaut de réponse aux signaux de danger au cours du syndrome VEXAS

Paul Breillat, Samuel J. Magaziner, Stéphane M. Camus, Léa Dionet et autres

fr, us (code pays fourni par la source)

UBA1 Mutations Drive RIPK1-Mediated Cell Death and Monocyte Dysfunction in VEXAS Syndrome

Paul Breillat, Samuel J. Magaziner, Stéphane M. Camus, Léa Dionet et autres

Abstract VEXAS syndrome is a severe adult-onset autoinflammatory disease caused by somatic mutations in UBA1 gene, disrupting cytoplasmic ubiquitin-activating enzyme E1 function in hematopoietic progenitors. The pathogenesis remains poorly understood, particularly how UBA1 mutations perturb myeloid function. Here, we combine a genetically …

fr, us, ca (code pays fourni par la source)