Fig. 2: Evaluation of immune cells in dysferlinopathic mice.

Representative FACS analysis of thymic subpopulations of 12 m C57Bl and 12 m BlAJ (A) and of DN stages (B). The amount of positively selected TCRβ+ CD69+ T cells and Tregs of 12 m C57Bl and 12 m BlAJ thymus (C). Spleen weight (in grams) in 12 m C57Bl and 12 m BlAJ mice (D). Representative FACS profile showing the percentage of each population of live cells: a gate of GR1-positive and F4/80 negative represents monocytes, neutrophils and granulocytes, further divided according to the expression of CD11b (E). Evaluation of spleen-derived granulocytes, monocytes and macrophages (F). FACS analysis of spleen-derived IFNγ- and IL17/IFNγ-producing CD4+ and CD8+ T cells (G) and of CD4+ and CD8+ T cells (H). Representative FACS profile is shown. The numbers within the panels indicate the percentage of each population of live cells: within the gate of CD4-positive or CD8-positive, two populations are separated by level of CD44 and CD62L expression (I). Evaluation of T-cell subsets—naive, central memory (CM), effector memory (EM)—expressing CD4 and/or CD8 in the spleens of 12 m C57Bl and 12 m BlAJ (J). Representative FACS profile showing the percentage of each population of live cells. Within the B220+ gate, two populations are separated by expression level of CD21 and CD23 (K). FACS analysis of total, follicular (FO, CD23+) and marginal zone (MZ, CD21+) splenic B cells of 12 m C57Bl and 12 m BlAJ mice (L). Representative FACS profile is shown. The numbers within the panels indicate the percentage of each population of live cells. Subpopulations are separated according to B220 and CD19 expression (M). FACS analysis of the percentage of mature B-cells (M) and evaluation of transitional T1 B (T1 B) and transitional T2 B (T2 B) splenic B cells of 12 m C57Bl and 12 m BlAJ mice (N). Data are presented as mean ± SD of n = 3 independent experiments with n = 3–9 animals/group. Student t-test: **p < 0.01.