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. 2018 Nov 23;9:2545. doi: 10.3389/fimmu.2018.02545

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Copyright © 2018 van Schouwenburg, IJspeert, Pico-Knijnenburg, Dalm, van Hagen, van Zessen, Stubbs, Patel and van der Burg.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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CVID patients have increased VH4-34 and JH6 usage and an increased CDR3 length. Mean CDR3 length (A) the percentage JH6 (B) and VH4-34 usage (C), in naïve B-cells and IGHA and IGHG transcripts of antigen selected B-cells in all samples or samples of which paired data was available (D–F). The dotted lines in (A–C) represent the mean ± the st dev of the controls. (G) Heatmap showing VH4-34 and JH6 usage and median CDR3 length for all patients. Values >2 st dev below the mean are depicted in increasingly darker shades or red, while values >2 st dev above the mean are depicted in increasingly darker shades of green. Patients are clustered based on the data included in the heatmap. CVID patients with autoimmunity are indicated in blue. Statistical significance is performed using a Mann Whitney-test and indicated using *P < 0.05, **P < 0.01 and ***P < 0.001.