Fig. 12.

Hoxb7 cre-lox kidney-specific conditional cilium-knockout (KO) mice with cystic kidneys and acid-base anomalies. A: a collecting duct from a 7-day old Ift88^flox/Δ; Hoxb7-cre mutant mouse displays a dilated tubule lumen and no cilium labeling with an antibody to acetylated α-tubulin (green). Nuclei are labeled with Hoechst (blue). B: a collecting duct from a 7-day old Ift88^WT/flox; Hoxb7-cre control mouse displays cilium labeling with the acetylated α-tubulin (green) antibody. Nuclei are labeled with Hoechst (blue). C: cystic lesions are evident in cross sections of paraffin-embedded whole kidney from a 14-day-old mutant mouse. D: cystic lesions are absent in cross sections of paraffin-embedded whole kidney from a 14-day-old control mouse. E: fresh urine samples (left) and blood samples (right) were collected from 50 Hoxb7-cre; Ift88^flox/Δ kidney-specific conditional cilium-knockout mice versus 116 littermate controls at postnatal days 7, 14, 19–23, and 28. Horizontal bars in the scatterplots represent mean pH values. *P < 0.01 (nonparametric Mann-Whitney t-test).