American Society of Hematology

Figure 4.

$Figure 4. Increased baseline expression of lysosomal marker proteins on T cells and lack of up-regulation upon stimulation. Expression of CD107 (A) and CD63 (B) on resting CD3+CD8+ T cells. Bold lines represent patient; thin lines, control T cells; and dashed line, staining with an isotype control antibody. (C-D) Degranulation of activated T cells assessed by CD107 expression. Short-term PHA blasts (36h) (C) or fresh PBMCs (D) were stimulated with anti-CD3/anti-CD28 beads in the presence of an antibody to CD107 followed by surface staining for CD3 and CD8 and subsequent intracellular staining for IFN-γ. Cells were analyzed by 4-color flow cytometry. Dot plots were gated on CD3+CD8+ cells (C) or on CD3+ cells (D) from healthy control donors (left column), the patient, and 2 patients with genetically confirmed GSII and CHS (right column). The numbers indicate the fraction of degranulating (CD107+) cells among the activated (IFN-γ producing) T cells.$

Increased baseline expression of lysosomal marker proteins on T cells and lack of up-regulation upon stimulation. Expression of CD107 (A) and CD63 (B) on resting CD3⁺CD8⁺ T cells. Bold lines represent patient; thin lines, control T cells; and dashed line, staining with an isotype control antibody. (C-D) Degranulation of activated T cells assessed by CD107 expression. Short-term PHA blasts (36h) (C) or fresh PBMCs (D) were stimulated with anti-CD3/anti-CD28 beads in the presence of an antibody to CD107 followed by surface staining for CD3 and CD8 and subsequent intracellular staining for IFN-γ. Cells were analyzed by 4-color flow cytometry. Dot plots were gated on CD3⁺CD8⁺ cells (C) or on CD3⁺ cells (D) from healthy control donors (left column), the patient, and 2 patients with genetically confirmed GSII and CHS (right column). The numbers indicate the fraction of degranulating (CD107⁺) cells among the activated (IFN-γ producing) T cells.

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