NDLI: Antibody deficiency in patients with ataxia telangiectasia is caused by disturbed B- and T-cell homeostasis and reduced immune repertoire diversity.

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Antibody deficiency in patients with ataxia telangiectasia is caused by disturbed B- and T-cell homeostasis and reduced immune repertoire diversity.

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Antibody deficiency in patients with ataxia telangiectasia is caused by disturbed B- and T-cell homeostasis and reduced immune repertoire diversity.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Driessen, Gertjan J. Ijspeert, Hanna Weemaes, Corry M. R. Haraldsson, Ásgeir Trip, Margreet Warris, Adilia Van Der Flier, Michiel Wulffraat, Nico Verhagen, Mijke M. M. Taylor, Malcolm A. Van Zelm, Menno C. Van Dongen, Jacques J. M. Van Deuren, Marcel Van Der Burg, Mirjam
Description	Country affiliation: Netherlands Author Affiliation: Driessen GJ ( Department of Pediatric Infectious Disease and Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.)
Abstract	BACKGROUND: Ataxia telangiectasia (AT) is a multisystem DNA-repair disorder caused by mutations in the ataxia telangiectasia mutated (ATM) gene. Patients with AT have reduced B- and T-cell numbers and a highly variable immunodeficiency. ATM is important for V(D)J recombination and immunoglobulin class-switch recombination (CSR); however, little is known about the mechanisms resulting in antibody deficiency severity. OBJECTIVE: We sought to examine the immunologic mechanisms responsible for antibody deficiency heterogeneity in patients with AT. METHODS: In this study we included patients with classical AT plus early-onset hypogammaglobulinemia (n = 3), classical AT (n = 8), and variant AT (late onset, n = 4). We studied peripheral B- and T-cell subsets, B-cell subset replication history, somatic hypermutation frequencies, CSR patterns, B-cell repertoire, and ATM kinase activity. RESULTS: Patients with classical AT lacked ATM kinase activity, whereas patients with variant AT showed residual function. Most patients had disturbed naive B-cell and T-cell homeostasis, as evidenced by low cell numbers, increased proliferation, a large proportion CD21(low)CD38(low) anergic B cells, and decreased antigen receptor repertoire diversity. Impaired formation of T cell-dependent memory B cells was predominantly found in patients with AT plus hypogammaglobulinemia. These patients had extremely low naive CD4(+) T-cell counts, which were more severely reduced compared with those seen in patients with classical AT without hypogammaglobulinemia. Finally, AT deficiency resulted in defective CSR to distal constant regions that might reflect an impaired ability of B cells to undergo multiple germinal center reactions. CONCLUSION: The severity of the antibody deficiency in patients with AT correlates with disturbances in B- and T-cell homeostasis resulting in reduced immune repertoire diversity, which consequently affects the chance of successful antigen-dependent cognate B-T interaction.
File Format	HTM / HTML
ISSN	00916749
e-ISSN	10976825
Journal	Journal of Allergy and Clinical Immunology
Issue Number	5
Volume Number	131
Language	English
Publisher	Elsevier
Publisher Date	2013-05-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Discipline Immunology Ataxia Telangiectasia Complications Immunology B-lymphocyte Subsets Homeostasis Immunologic Deficiency Syndromes Etiology T-lymphocyte Subsets Adolescent Agammaglobulinemia Genetics Case-control Studies Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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