Delayed cystogenesis and increased ciliogenesis associated with the re-expression of polaris in Tg737 mutant mice  Nicole E. Brown, Noel S. Murcia  Kidney International  Volume 63, Issue 4, Pages 1220-1229 (April 2003) DOI: 10.1046/j.1523-1755.2003.00863.x Copyright © 2003 International Society of Nephrology Terms and Conditions

Figure 1 Serial sections localizing morphology and segment identity. Postnatal day (P)50 KO rescue (A and B) and P50 orpk rescue (C and D) kidney sections (×200) showing proximal tubules (PT) [biotin-labeled Lotus Tetragonobolus (LTA) positive (A) and (C) and collecting tubules (CT) biotin-labeled Dolichos biflorus agglutinin (DBA) positive (B) and (D) exhibiting mild dilations. Asterisks in (A) highlight examples of LTA-positive PTs and asterisks in (B) highlight examples of DBA-positive CTs. P150 KO rescue (E and F) and P150 orpk rescue (G and H) kidney sections ×200) showing that PTs (E) and (G) and CTs (F) and (H) exhibit numerous cystic lesions at this age. Asterisks in (E) highlight examples of PT cysts and asterisks in (F) highlight examples of CT cysts. Note that cystic disease is slightly more severe in orpk rescue (G and H) than KO rescue (E and F). P150 control animals exhibit normal PT (I and K) and CT (J and L) morphology for this age (×200). P14 control (M and N) (×200) and orpk mutant (O and P) (×100) kidney sections demonstrate the early onset PT cysts (O, asterisk) and CT cysts (P, asterisk) normally associated with the orpk mutation. Kidney International 2003 63, 1220-1229DOI: (10.1046/j.1523-1755.2003.00863.x) Copyright © 2003 International Society of Nephrology Terms and Conditions

Figure 2 Ultrastructural analysis of cilia expression in renal cysts from KO rescue,orpk rescue, andorpk mutants animals. Postnatal day (P)200 KO rescue renal cysts showing a low magnification view (A) (×50), a high magnification view of a typical PT brush border cyst (B) (×4000), and a high magnification view of a typical nonbrush-border cyst (C) (×4000). Note that cilia are not visible above the brush border (BB) in (B) and short cilia are visible in (C). P200 orpk rescue renal cysts showing a low magnification view (D) (×40), a high magnification view of a cyst located near the edge of the PT brush border (E) (×4300), and a high magnification view of a typical nonbrush-border cyst (F) (×5000). Note that long cilia are prominently visible in both types of cysts. P14 orpk mutant kidney showing a low magnification view (G) (×100) and high magnification views of four different types of cysts. Cysts expressing 1 to 2 μ cilia (H) (×8000), 3 to 4 μm cilia (I) (×8000), multiple cilia (J) (×12000), and no cilia (K) (×3500) were present in orpk mutant kidneys. These types of cysts were also found in KO rescue and orpk rescue cysts at different frequencies (see Table 2). P200 KO/+;Tg737Rsq control kidney tubule (L) exhibiting normal morphology and 1 to 2 μm cilia consistent with wild-types of this age. Bars, (A), (D), and (G) 100 μm; (B), (C), (E), (F), and (H) to (L) 1μm. Kidney International 2003 63, 1220-1229DOI: (10.1046/j.1523-1755.2003.00863.x) Copyright © 2003 International Society of Nephrology Terms and Conditions