Article

Pathogenic mechanisms underlying immune dysregulation associated to hypomorphic Rag2 mutation : role of microbiota at mucosal interfaces

2018-01-01

Abstract excerpt

Hypomorphic mutations in the <i>RAG</i> genes cause a profound immunodeficiency associated with multisystem autoimmune–like manifestations, mediated by oligoclonal self-reactive T and B cells, named Omenn Syndrome (OS). The clinical and immunological spectrum of OS presentation is extremely broad. Commonly, patients present symptoms very similar to graft-versus-host disease (GVHD) as inflammatory reactions particu...

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Literature Corpus work: ac611904-c1bd-5c0b-af80-305c3133948c
DOI: 10.21954/ou.ro.0000d6c1

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