Figure 4

Ciliogenesis defects in NPHP5 URECs are rescued by Alprostadil/PGE1. (a) Immunofluorescence for primary cilia (ARL13B, green) and basal bodies markers (γ- tubulin, red) of NPHP5 URECs (2.05P1, 2.05P2) treated with 0.04% DMSO or 2 μM alprostadil (Alpro) for 48 hours. Scale bar 10 μm. (b) Ciliogenesis was quantified in control (1.56NC), NPHP5 (2.05P1, 2.05P2) and NPHP1 (1.12P) URECs treated with either DMSO (0.04%) or 2 μM alprostadil (Alpro), were fixed and stained similarly as in (a) (n = 4 experiments). Mixed linear regression with quasi-binomial penalization: ∗∗∗P < 0.001, ∗∗P < 0.01, ∗P < 0.05. (c) Cilium length was quantified as in Figure 1 in control (1.56NC) and NPHP5 (2.05P1, 2.05P2) URECs treated with 0.04% DMSO (-) or 2μM alprostadil (Alpro). n = 4 experiments; paired 2-tailed t: ∗∗∗∗P < 0.0001, ∗∗∗P < 0.001, ∗∗P < 0.01. DMSO, dimethyl sulfoxide; UREC, urinary renal epithelial cells.