Figure 6

Cyst formation in kat2J mice. (A) Cysts in kat2J/Nek1 −/− kidneys form in different parts of the nephron and from multiple tubular subtypes. Representative H&E-stained sections of a kidney from a 1-day-old mouse (a, original magnification 40x) and 21-day-old mouse (b, original magnification 40x; bar, 100 μm) show tubular and glomerular cysts. (c-f) Immunohistochemical staining for proximal tubules with Lotus tetragonolobus lectin (c, LTL), loops of Henle with Tamm-Horsfall glycoprotein (e, THP), collecting tubules with Dolichos biflorus lectin (d, DBF), and other collecting ducts with aquaporin-2 (f, AQP2). Bar, 100 μm. (B) Cystic disease in kat2J/Nek1+/− mice. (a, b) Cysts develop focally in kat2J/Nek1+/− old adult mice (age 415 days). (c, d) Different sections from the same aged different mouse show patchy upregulation of Nek1 in some tubules (arrows) but loss of Nek1 staining in cystically dilated tubules (asterisks). (e, f) In representative sections from a kat2J/Nek1 +/− littermate, Nek1 expression is upregulated in some tubules but minimal or absent in other, cystically dilated tubules. Bar, 100 μm for all panels.