Mechanisms of hematopoietic clonal dominance in VEXAS syndrome.

doi:10.1038/s41591-025-03623-9

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Article

Mechanisms of hematopoietic clonal dominance in VEXAS syndrome.

Nature medicine - 1 Jun 2025

Molteni Raffaella, Fiumara Martina, Campochiaro Corrado, Alfieri Roberta, Pacini Guido, Licari Eugenia, Tomelleri Alessandro, Diral Elisa, Varesi Angelica, Weber Alessandra, Quaranta Pamela, Albano Luisa, Gaddoni Chiara, Basso-Ricci Luca, Stefanoni Davide, Alessandrini Laura, Degl'Innocenti Sara, Sanvito Francesca, Bergonzi Gregorio Maria, Annoni Andrea, Panigada Maddalena, Cantoni Eleonora, Canarutto Daniele, Xie Stephanie Z, D'Alessandro Angelo, Di Micco Raffaella, Aiuti Alessandro, Ciceri Fabio, De Luca Giacomo, Dagna Lorenzo, Matucci-Cerinic Marco, Merelli Ivan, Cenci Simone, Scala Serena, Cavalli Giulio, Naldini Luigi, Ferrari Samuele

Abstract excerpt

Clonal dominance characterizes hematopoiesis during aging and increases susceptibility to blood cancers and common nonmalignant disorders. VEXAS syndrome is a recently discovered, adult-onset, autoinflammatory disease burdened by a high mortality rate and caused by dominant hematopoietic clones bearing somatic mutations in the UBA1 gene. However, pathogenic mechanisms driving clonal dominance are unknown....

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Identifiers

PMID: 40195449
DOI: 10.1038/s41591-025-03623-9
PMCID: PMC12176642

Genes

UBA1

Provenance

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