Jos Jansen

3 85 TMEM199 Deficiency is characterized by elevated liverenzymes, hypercholesterolemia and abnormal glycosylation Table S2 quantifications of Apolipoprotein C-III Isoelectric focusing 0-sialo (%) 1-sialo (%) 2-sialo (%) Control range Age 0-1 0.2-4.5 42.7-69.8 26.2-56.7 Age 1-18 1.4-9.5 48.5-75.2 21.0-45.8 Age > 18 1.8-7.6 47.4-73.6 20.2-46.5 Family individual Age at analysis F1 II2 17 5.1 83.9 ↑ 11.0 ↓ II3 9 5.9 81.7 ↑ 12.6 ↓ F2 II2 > 18 7.2 65.5 27.3 F3 II1 12 3.5 85.3 ↑ 11.2 ↓ Table S3 Glycan structures (peak numbers associated with Figure 3C) Peak Structure Predicted mass (amu) 1 79555 2 79265 3 79104 4 78973 5 78811 6 78649

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