Figure 6.

Quantification and scoring of Alcian Blue, LAMP1 and CD68 in peripheral organs. (A–G) Scoring of Alcian Blue in peripheral organs and analysis of cartilage thickness. Scoring of Alcian Blue in liver (A), spleen (B), kidney (C), heart valves (F), and heart vessels (G). Scoring rules for Alcian Blue staining are shown in Supplementary Table S1 (liver, spleen, and kidney), Supplementary Table S2 (heart valves) and Supplementary Table S3 (great heart vessels). Cartilage thickness was measured in the growth plate of the distal epiphysis of the femur (D) and in trachea (E). (H–O) Scoring and quantification of LAMP1 pathology. Scoring of LAMP1 pathology in liver (H), spleen (I), and kidney (J). Scoring rules for LAMP1 staining are shown in Supplementary Table S4 (liver and spleen) and Supplementary Table S5 (kidney). Quantification of LAMP1 signal in the growth plate of the distal epiphysis of the femur (K), articular knee cartilage (L), trachea (M), heart valves (N), and in great heart vessels (O). Scoring of CD68 pathology in liver (P) and kidney (Q) using the scoring rules shown in Supplementary Table S6 (liver and kidney). Data represent means ± SEM and were analyzed by one-way ANOVA followed by Bonferroni's multiple testing correction. *p ≤ 0.05, **p ≤ 0.01, ***p ≤ 0.001, ****p ≤ 0.0001. ^#p ≤ 0.05, ^##p ≤ 0.01, ^###p ≤ 0.001, ^####p ≤ 0.0001. Asterisks represent significance versus WT; hash represent significance versus Ids^y/−. Significant results are indicated by brackets.