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Human IκBα gain of function: a severe and syndromic immunodeficiency

Germline heterozygous gain-of-function (GOF) mutations of NFKBIA, encoding IκBα, cause an autosomal dominant (AD) form of anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID). Fourteen unrelated patients have been reported since the identification of the first case in 2003. All mutations e...

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Detalles Bibliográficos
Publicado en:J Clin Immunol
Main Authors: Boisson, Bertrand, Puel, Anne, Picard, Capucine, Casanova, Jean-Laurent
Formato: Artigo
Idioma:Inglês
Publicado: 2017
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC5563390/
https://ncbi.nlm.nih.gov/pubmed/28597146
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s10875-017-0400-z
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