Impaired Antibody Affinity Maturation Process Characterizes a Subset of Patients with Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is an heterogeneous syndrome characterized by decreased levels of serum Ig and recurrent bacterial infection. Here, we were interested to study whether a qualitative defect of the affinity Ab maturation process could be combined to the low level of serum Ig in...

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Bibliographic Details
Published in:The Journal of immunology (1950) 2000-10, Vol.165 (8), p.4725-4730
Main Authors: Bonhomme, D, Hammarstrom, L, Webster, D, Chapel, H, Hermine, O, Le Deist, F, Lepage, E, Romeo, P. H, Levy, Yves
Format: Article
Language:English
Subjects:
Adult
Aged
Alanine - genetics
Antibody Affinity - genetics
B-Lymphocyte Subsets - immunology
B-Lymphocyte Subsets - metabolism
Clone Cells
Codon
Common Variable Immunodeficiency - blood
Common Variable Immunodeficiency - genetics
Common Variable Immunodeficiency - immunology
Consensus Sequence
DNA Mutational Analysis
DNA-Binding Proteins - blood
DNA-Binding Proteins - genetics
Genes, Immunoglobulin
Humans
Immunoglobulin Constant Regions - genetics
Immunoglobulin G - genetics
Immunoglobulin Heavy Chains - genetics
Immunoglobulin Variable Region - genetics
Immunologic Memory - genetics
Medicin och hälsovetenskap
Middle Aged
Mutation
Proto-Oncogene Proteins - blood
Proto-Oncogene Proteins - genetics
Proto-Oncogene Proteins c-bcl-6
Serine - genetics
Transcription Factors - blood
Transcription Factors - genetics
Transcription, Genetic - immunology
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Summary:Common variable immunodeficiency (CVID) is an heterogeneous syndrome characterized by decreased levels of serum Ig and recurrent bacterial infection. Here, we were interested to study whether a qualitative defect of the affinity Ab maturation process could be combined to the low level of serum Ig in a cohort of 38 CVID patients. For this, we designed a novel and rapid screening test for the detection of hypomutated V gene expressed by memory B cells. This test delineated a subset of 9/38 (23%) CVID patients with an abnormal pattern of Ig V gene mutation. The mean frequency of V gene mutation of this subset was significantly lower (1.74%) compared with other CVID patients (5.46%) and normal donors (6.5%) (p
ISSN:0022-1767
1550-6606
DOI:10.4049/jimmunol.165.8.4725