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Supplemental Information Table 1. Left ventricular size and function in MCM;Myc+/+ and MCM;Mycfl/fl mice after 2 weeks of Sham or TAC MCM;Myc+/+ MCM;Mycfl/fl Sham TAC Sham TAC 6 6 6 6 HR (bpm) 506+20 540+29 502+36 527+17 ESD (mm) 1.75+0.15 1.95+0.24 1.68+0.14 1.80+0.15 EDD (mm) 3.18+0.12 3.21+0.18 3.20+0.12 3.13+0.17 IVST (mm) 0.69+0.06 0.93+0.08* 0.72+0.03 0.79+0.04 PWT (mm) 0.65+0.06 0.81+0.1 0.68+0.06 0.69+0.08 AoEt (ms) 55.8+3.2 59.4+1.3 56.7+2.0 57.6+1.9 LV %FS 45.3+3.2 40.5+3.7 47.8+2.4 42.7+2.7 Vcf (mm/s) 8.29+0.84 6.81+0.61 8.49+0.60 7.49+0.61 LVM (mg) 60.2+5.8 88.1+8.5* 64.6+4.1 68.3+8.2 n Echocardiographic analysis of the left ventricle: HR, heart rate; ESD, end-systolic diameter; EDD, end-diastolic diameter; IVST, intraventricular septal thickness; PWT, posterior wall thickness; AoET, aortic ejection time; LV%FS, left ventricular fractional shortening; Vcf, circumferential shortening velocity; LVM, calculated left ventricular mass. Results presented as mean+S.E.M., ANOVA *p<0.05 versus Sham animals Table 1 – 2-D echocardiograms to assess LV function were performed on 4-OHT-treated control MCM:Myc+/+ or MCM:Mycfl/fl mice after two weeks of Sham or TAC surgery. 2-D Echo studies. Echocardiography on transgenic mice was performed as described (Jordan et al, 2005). Estimation of left ventricular mass has been described (Gardin et al, 1995). At least three independent M-mode measurements per animal were obtained by an examiner blinded to the genotype of the animal. References Gardin JM, Siri FM, Kitsis RN, Edwards JG, Leinwand LA (1995) Echocardiographic assessment of left ventricular mass and systolic function in mice. Circ Res 76: 907-914. Jordan MC, Zheng Y, Ryazantsev S, Rozengurt N, Roos KP, Neufeld EF (2005) Cardiac manifestations in the mouse model of mucopolysaccharidosis I. Mol Genet Metab 86: 233-243.