Fig. 4
From: Novel pathogenic variants in CUBN uncouple proteinuria from renal function
Dysfunction of Cubilin induced by the variants locating after vitB12-binding domain of CUBN was accompanied by abnormal localization of AMN. A The protein expression of Cubilin and Megalin in kidney tissues was detected by immunofluorescence assay and showed the expression and localization of Megalin did not accompany the decrease of Cubilin expression. B Immunofluorescence staining showed the decrease expression of Cubilin caused by the CUBN variant was accompanied by aberrant cytoplasmic localization of AMN in renal tubule membrane. C and D Representative photomicrographs by TEM analyses confirmed the GFB damage and immunofluorescence staining were to detect the colocalization and expression between Cubilin and AMN in kidney biopsies from MCD and FSGS. E and F The reduced expression of renal Cubilin was not accompanied by abnormal localization of AMN in the mice model of LPS-induced acute kidney injury and Adriamycin-induced nephropathy. Each group was tested in triplicate, and the data are given data are presented as the mean ± S.D. ns, no significant; *P < 0.05; **P < 0.01; ***P < 0.001