Panel A shows flow cytometry plots comparing CXCR5-positive PD-1-positive circulating follicular helper T-cell frequencies across study groups. Panel B shows a bar graph quantifying frequencies of CXCR5-positive PD-1-positive circulating follicular helper T cells. Panel C shows histograms and bar graphs comparing PD-1 expression levels on circulating follicular helper T cells. Panel D shows flow cytometry plots illustrating circulating follicular helper T-cell subset distributions based on chemokine receptors. Panel E shows bar graphs quantifying type 1, type 2, and type 17 circulating follicular helper subsets. Panel F shows flow cytometry plots depicting naïve, central memory, effector memory, and EMRA T-cell compartments. Panel G shows bar graphs summarizing memory and naïve T-cell subset distributions within CD4-positive and CD8-positive populations. Panel H shows bar graphs comparing frequencies of recent thymic emigrants within conventional and regulatory T-cell populations. Panel I shows flow cytometry plots illustrating CD39 expression within CD4-positive FOXP3-negative conventional T cells. Panel J shows flow cytometry plots comparing Helios expression within CD4-positive FOXP3-negative conventional T cells. Panel K shows bar graphs quantifying frequencies of CD39-positive and Helios-positive conventional T-cell populations. Panel L shows flow cytometry plots illustrating interferon-gamma-producing CD4-positive T-cell frequencies across study groups. Panel M shows bar graphs comparing frequencies of cytokine-producing CD4-positive and CD8-positive T-cell populations.
Abatacept restores T cell homeostasis disrupted by the CTLA4 S172P/S172P variant. (A) Representative flow cytometry plots of CXCR5+PD-1+ cTFH cells comparing HCs, mother, and patient before and after abatacept treatment (12 mo). (B) Percentages of CXCR5+PD-1+ cTFH cell frequencies across HC, mother, and patient before and after abatacept (12 mo). (C) Representative histograms showing PD-1 MFI on CXCR5+ cTFH cells, with bar graphs summarizing MFI values in HC, mother, and patient before and after abatacept (abt) (12 mo). (D) Representative plots depicting cTFH subset distribution based on TH1 (CXCR3+CCR6−), TH2 (CXCR3−CCR6−), and TH17 (CXCR3−CCR6+) phenotypes in all donors. (E) Bar graphs showing the percentages of TH1, TH2, and TH17 cTFH subsets in HC, mother, and patient before and after abatacept therapy. (F) Representative plots illustrating CD4+FOXP3− Tcon cell differentiation into naïve (CD45RA+CCR7+), central memory (CD45RA−CCR7+), effector memory (CD45RA−CCR7−), and EMRA (CD45RA+CCR7−) compartments across the donors. (G) Bar graphs depicting the percentages of naïve, central memory, effector memory, and EMRA CD4+FOXP3− Tcon and CD8+ T cells in HC, mother, and patient before and after abatacept. (H) Frequencies of recent thymic emigrants (CD3+CD45RA+CD31+) within CD4+FOXP3− Tcon and CD4+FOXP3+ Tregs across all donors. (I) Representative plots of CD39 expression within CD4+FOXP3− Tcon cells in HC, mother, and patient before and after treatment. (J) Representative plots showing Helios expression in CD4+FOXP3− Tcon cells across donors and treatment conditions. (K) Bar graphs showing the percentages of CD39+ and Helios+ Tcon cells in HC, mother, and patient before and after abatacept. (L) Representative flow cytometry plots of IFN-γ+ CD4+ T cells across HC, mother, and patient before and after abatacept. (M) Bar graphs showing the proportions of cytokine-producing T cells (CD4+IFN-γ+, CD4+IL-10+, CD4+IL-4+, CD4+IL-17A+, and CD8+IFN-γ+) in HC, mother, and patient before and after abatacept. HC, healthy control, MFI, mean fluorescence intensity.