Morphologic and functional characteristics of Col4a1 G498V mutant adult kidney. (A) Histologic analysis of 6-month adult kidney. Heterozygous (+/G498V) and homozygous (G498V/G498V) mice developed cystic glomeruli with dilation of Bowman’s space and retracted capillary tuft (first column, Masson’s trichrome). Scale bars: 200 µm. Noncystic glomeruli showed cuboidal PECs (black arrows) (second column, Jones’ methenamine silver). Nodular inflammatory infiltrates were seen in both heterozygous and homozygous mutant kidneys around glomeruli (third column) and arterioles (fourth column) (Masson’s trichrome). Scale bar: 50 µm. (B) Transverse sections of 12-month heterozygous and homozygous kidneys showing distribution of glomerular cysts throughout the cortex area. Note the dysmorphic papilla in homozygous kidney (Masson’s trichrome). Scale bar: 1 mm. (C) Glomeruli with cystic change (cyst surface, left panel: 11,283 μm2; cyst surface, right panel: 14,900 μm2) showing a normal glomerulotubular junction (black arrow) in +/G498V and G498V/G498V 6-month kidneys (periodic acid–Schiff). Scale bar: 20 μm. (D) Percentage of glomeruli with cystic changes (left panel) and glomerular cyst area (right panel) in heterozygous and homozygous kidneys at 3 months (+/G498V, n=4; G498V/G498V, n=4), 6 months (+/G498V, n=8; G498V/G498V, n=6) and 12 months (+/G498V, n=7; G498V/G498V, n=7) of age. Values represent mean±SEM. *P<0.05, ***P<0.001.