Figure 1

Representative images of kidneys from 1 month-old TgUmodC147W and TgUmodwt mice. Immunohistochemistry analysis for total uromodulin shows that the protein is mainly distributed at the apical membrane of TAL cells in TgUmodwt mice, while it is intracellularly enriched in TgUmodC147W mice (left panels, scale bar 100 µm). Representative images of kidney sections (right panels, PAS, scale bar 200 µm) and quantification of histological parameters (bottom) are shown. Data are expressed as mean ± s.d. (n = 9 TgUmodwt and 6 TgUmodC147W). TgUmodC147W mice show a relatively well preserved kidney structure. Only tubular casts, mostly uromodulin-positive (left panel, arrowhead) resulted to be increased. **P < 0.01 (Mann-Whitney test).