Fig 1: Hoil-1lnzf*/nzf; Sharpincpdm/cpdm mice show systemic inflammation and a distinctive immune cell composition(A) Numbers of weaned and expected pups of the indicated genotypes from Hoil-1l+/nzf*; Sharpin+/cpdm crosses.(B) Representative image of the gross appearance from 2-week-old mice of the indicated genotypes. Scale bar: 10 mm.(C) Body weight of 4-week-old mice from the indicated genotypes.(D) Representative images of the spleen of indicated genotype. Scale bar: 10 mm.(E) Spleen weight of 4-week-old mice from the indicated genotypes.(F) H&E staining, CD45R and CD3 immunostaining of spleen sections from 4-week-old mice of the indicated genotypes. Scale bar: 50 µm.(G) Percentage of viable myeloid cells (CD11b+), normalized to the frequency of viable CD45+ cells, in the spleen from 4-week-old mice of the indicated genotypes.(H) Percentage of neutrophils (CD11bHi Ly6G+) out of viable CD45+ cells in the spleen from 4-week-old mice of the indicated genotypes.(I and J) Percentage of inflammatory monocytes (CD11c- CD11b+ Ly6G- Ly6C+) (I) and conventional monocytes (CD11c- CD11b+ Ly6G- Ly6C-) (J), out of viable CD45+ CD11b+ cells, in the spleen from 4-week-old mice of the indicated genotypes.(K) Percentage of plasma cells (CD138+ CD28+), out of viable B220+ Lin- cells, in the mesenteric lymph node (mLN) from 4-week-old mice of the indicated genotypes. The lineage (Lin) cocktail consists of TCRß, DX5, CD11b, and CD23. Data are represented as mean ± SD. ANOVA, **p value=0.01, ***p value=0.001, ****p value =0.0001. (C and E) Hoil-1l+/+; Sharpin+/+; TnfrI+/+ (n = 13 for C, n = 15 for E), Hoil-1lnzf*/nzf*; Sharpin+/+;TnfrI+/+ (n = 10), Hoil-1l+/+; Sharpincpdm/cpdm ;TnfrI+/+ (n = 11 for C, n = 10 for E), Hoil-1l+/nzf*; Sharpincpdm/cpdm;TnfrI+/+ (n = 25), Hoil-1lnzf*/nzf*;Sharpincpdm/cpdm;TnfrI+/+ (n = 9 for C, n = 7 for E), Hoil-1lnzf*/nzf*; Sharpincpdm/cpdm; TnfrI-/- (n = 5) (G-K) Hoil-1l+/+; Sharpin+/+ (n = 8 for G-J, n = 6 for K), Hoil-1lnzf*/nzf*; Sharpin+/+ (n = 6 for G-J, n = 7 for K), Hoil-1l+/+; Sharpincpdm/cpdm (n = 6), Hoil-1l+/nzf*; Sharpincpdm/cpdm (n = 9 for G-J, n = 8 for K), Hoil-1lnzf*/nzf*; Sharpincpdm/cpdm (n = 4).