B-cell development and functions and therapeutic options in adenosine deaminase–deficient patients

BACKGROUND: Adenosine deaminase (ADA) deficiency causes severe cellular and humoral immune defects and dysregulation because of metabolic toxicity. Alterations in B-cell development and function have been poorly studied. Enzyme replacement therapy (ERT) and hematopoietic stem cell (HSC) gene therapy...

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Publicat a:J Allergy Clin Immunol
Autors principals: Brigida, Immacolata, Sauer, Aisha V., Ferrua, Francesca, Giannelli, Stefania, Scaramuzza, Samantha, Pistoia, Valentina, Castiello, Maria Carmina, Barendregt, Barbara H., Cicalese, Maria Pia, Casiraghi, Miriam, Brombin, Chiara, Puck, Jennifer, Müller, Klaus, Notarangelo, Lucia Dora, Montin, Davide, van Montfrans, Joris M., Roncarolo, Maria Grazia, Traggiai, Elisabetta, van Dongen, Jacques J. M., van der Burg, Mirjam, Aiuti, Alessandro
Format: Artigo
Idioma:Inglês
Publicat: 2014
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4489526/
https://ncbi.nlm.nih.gov/pubmed/24506932
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jaci.2013.12.1043
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