Upregulated SCPs in MFS mice Upregulated SCPs in MFS patients

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Upregulated SCPs in MFS mice Upregulated SCPs in MFS patients -log10(p) B Upregulated SCPs in MFS patients -log10(p) Supplementary Figure 1

A B Supplementary Figure 2 Downregulated SCPs after baclofen treatment in CMap database – baclofen (experiment # 2036) -log10(p) B Downregulated SCPs after baclofen treatment in CMap database – baclofen (all experiments combined) -log10(p) Supplementary Figure 2

Supplementary Figure 3 A B Downregulated SCPs in WT mice +/- BAC -log10(p) B Downregulated SCPs in MFS mice +/- BAC -log10(p) Supplementary Figure 3

Supplementary Fig. 4 A 25 50 100 75 Percent Survival 30 60 90 25 50 100 75 Percent Survival 30 60 90 Days After Birth WT VEH MFS ENA MFS VEH MFS PRO ns B 0.00 0.05 0.10 0.15 0.20 0.25 0.00 0.05 0.10 0.15 0.20 0.25 0.30 AoR Diameter (cm) AsA Diameter (cm) WT MFS MFS MFS WT MFS MFS MFS VEH VEH PRO ENA VEH VEH PRO ENA Supplementary Fig. 4

Supplementary Fig. 5 A B C WT VEH MFS BAC Circumferential Stiffness (MPa) 2 4 6 8 WT VEH MFS BAC Circumferential Stress (kPa) 200 400 600 WT VEH MFS BAC Stored Elastic Energy (kPa) 50 100 150 Supplementary Fig. 5