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. 2014 Aug 7;9(10):1729–1736. doi: 10.2215/CJN.00920114

Figure 2.

Figure 2.

PKHD1 expression in kidney tissue. The top panel shows relative PKHD1 expression in moderately affected patient F730 compared with the expression pattern in WT and kidney tissue from a patient with a truncating PKHD1 mutation and a severe form of autosomal recessive polycystic kidney disease (F304). *P<0.05; **P<0.001 compared with WT. The bottom panel shows the results of Western blot (WB) analysis using an antibody against the C-terminal end of polyductin (anti-polyductin, IZ-4-rb1) in renal tissue in the moderately affected patient carrying the homozygous mutation c.2130_2131insTA (F730) compared with the expression of two different wild-type tissues (WT1 and WT2, each different from the one used for RT-PCR experiments) and kidney tissue from a perinatally demised patient carrying the homozygous PKHD1 deletion c.4256_4257del (p.Arg1419fs) (F304).