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Supplementary Fig. 1. A. †. Gout. †. Gout. Gout Renal failure. †. N.E. N.E. Gout Renal failure. Propositus. Gout. Hyperuricemia. Gout Renal failure. G A. Supplementary Fig. 1. B. Cys 112 Tyr. G 335 A. Exon2. Exon 3. Exon4. Exon 5-8. Leader Peptide. EGF-like. Cys-rich.
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Supplementary Fig. 1 A † Gout † Gout Gout Renal failure † N.E. N.E. Gout Renal failure Propositus Gout Hyperuricemia Gout Renal failure
GA Supplementary Fig. 1 B Cys112Tyr G335A Exon2 Exon 3 Exon4 Exon 5-8 Leader Peptide EGF-like Cys-rich Zona Pellucida CCS YY Y Y Y GPI EHP Y Y EGF1 cbEGF2 cbEGF3 D8C ZP-C ATG ZP-N 22 31 281 334 426 505 585 614 640 C112Y IHP
Supplementary Fig. 2 WT C112Y WT C112Y MG132 - - + + soluble 75KDa- uromodulin insoluble 75KDa-
Supplementary Fig. 3 WT C112Y Cell lysate Vector P + + Lactacystin - - Uromodulin 75KDa- GFP β-actin
Supplemental Fig.4 WT C112Y Topiroxostat (30μM) - + - + Uromodulin 75kDa- Secreted Uromodulin 75kDa- GFP β-acin
