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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Udgivet i:J Allergy Clin Immunol
Main Authors: 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
Sprog:Inglês
Udgivet: 2014
Fag:
Article
Online adgang: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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