The large size of the gene makes it a significant mutational target and a wide range of mainly private mutations have been described. Phenotype/genotype correlations indicate that two truncating mutations are associated with the severe, lethal phenotype, whereas one or two missense changes are often associated with milder disease.18 This phenotypic spread is also reflected in the available animal models of ARPKD with Pkhd1 mutations.19, 20 The polycystic kidney (PCK) rat with a frameshifting exon 36 skipping mutation8 and the ex40 mouse with an inframe deletion of exon 4020 develop liver cysts and fibrosis. In the kidney the PCK rat has distal tubule and CD cysts,19 but the ex40 mouse has been reported to have a histologically normal kidney up to 14 months of age.20.Take the recent Manchester derby by the way, is it over yet? In the first half, City stood toetotoe with their outoftown rivals. Bellamy was destroying Rio and Tevez should have scored from a simple chance. But United f