Defective Self-Reactive Antibody Repertoire of Serum IgM in Patients with Hyper-IgM Syndrome

作者: Luc Mouthon , Igor Resnick , Sébastien Lacroix-Desmazes , Hans Ochs , Teresa Espanol

DOI:

关键词: Immunoglobulin MImmunoglobulin GImmunologyStaphylococcus epidermidisAutoantibodyKidneyHyper IgM syndromeBiologyHypergammaglobulinemiaB cell

摘要: We have analyzed the self-reactive repertoires of IgM and IgG Abs in serum 19 patients with hyper-IgM syndrome (HIM) by means a quantitative immunoblotting technique that allows for comparison Ab health disease multiparametric statistical analysis. Normal tissue extracts liver, lung, stomach, kidney were used as sources self Ags. Extracts Pseudomonas aeruginosa Staphylococcus epidermidis nonself demonstrate significant bias reactivities HIM toward Ags did not differ, however, between controls. No difference was found untreated those receiving substitutive treatment i.v. IgG. lacked reactivity Ags, although it exhibited pattern similar to healthy The data functional CD40-CD40 ligand interactions are essential selection natural B cell repertoires.

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