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PMID: 23765059 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Review

Primary antibody deficiencies.

Nature reviews. Immunology ·Vol. 13 ·No. 7 ·2013-07-00 ·Pages 519-33

Durandy A, Kracker S, Fischer A

Abstract

Primary antibody deficiencies (PADs) are the most common inherited immunodeficiencies in humans. The use of novel approaches, such as whole-exome sequencing and mouse genetic engineering, has helped to identify new genes that are involved in the pathogenesis of PADs and has enabled the characterization of the molecular pathways that are involved in B cell development and function. Here, we review the different PADs in terms of their known or putative mechanisms, which can be B cell intrinsic, B cell extrinsic or not defined so far. We also describe the clinical manifestations (including susceptibility to infections, autoimmunity and cancer) that have been associated with the various PADs.

MeSH Terms
Animals B-Lymphocytes/immunology Humans Immunity, Innate/immunology Immunologic Deficiency Syndromes/genetics,immunology
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Durandy Anne
National Institute of Health and Medical Research, INSERM U768, Necker Children's Hospital, F-75015 Paris, France. anne.durandy@inserm.fr
Kracker Sven
Fischer Alain
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Article Info
Journal
Nature reviews. Immunology
Abbr.
Nat Rev Immunol
ISSN
1474-1741
DOI
10.1038/nri3466
Email
anne.durandy@inserm.fr
Published
2013-07-00
Epub
2013-00-14
Pages
519-33
Language
English
Region
England
NLM ID
101124169
Subset
IM
External Links
PubMed source DOI: 10.1038/nri3466 View journal details
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