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. Author manuscript; available in PMC: 2014 May 7.
Published in final edited form as: Nature. 2013 Oct 9;503(7474):126–130. doi: 10.1038/nature12614

Figure 4. Immunologic abnormalities in SSS mice are prevented by integrin-modulating therapies.

Figure 4

(A) Increased circulating levels of anti-nuclear and anti-topoisomerase I antibodies by enzyme-linked immunosorbent assay (ELISA) in Fbn1D1545E/+ mice at 3 months of age are normalized upon treatment with β1aAb but not an isotype-matched control (IgG). Isotype control-treated: n = 6 (Fbn1+/+), 4 (Fbn1D1545E/+); β1aAb-treated: n = 4 (Fbn1+/+), 10 (Fbn1D1545E/+). (B) The cells expressing high α5β1 integrin in the dermis of mutant mice are CD317(high) cells that fail to accumulate upon treatment with β1aAb but not an isotype-matched control (IgG). The CD317(high) cells that accumulate in the dermis of mutant mice are B220(+)CD3(−)CD19(−) plasmacytoid dendritic cells and (C) express both IFNα and IL-6. For panels B–C: Isotype control-treated: n = 5 (Fbn1+/+), 7 (Fbn1D1545E/+); β1aAb-treated: n = 4 (Fbn1+/+), 7 (Fbn1D1545E/+). For panel E: n = 5 (Fbn1+/+), 4 (Fbn1D1545E/+), 4 (Fbn1DW1572C/+). DE = D1545E. WC = W1572C. * p<0.05, ** p<0.01, † p<0.001, ‡ p<0.0001.