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A RAG1 mutation found in Omenn Syndrome causes coding flank hypersensitivity: a novel mechanism for antigen receptor repertoire restriction

Hypomorphic RAG mutants with severely reduced V(D)J recombination activity cause Omenn Syndrome (OS), an immunodeficiency with features of immune dysregulation and a restricted T cell receptor repertoire. Precisely how RAG mutants produce autoimmune and allergic symptoms has been unclear. Current mo...

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Autors principals: Wong, Serre-Yu, Lu, Catherine P., Roth, David B.
Format: Artigo
Idioma:Inglês
Publicat: 2008
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2597290/
https://ncbi.nlm.nih.gov/pubmed/18768869
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