In the CHF mouse model, fibrocystin-defective cholangiocytes possess increased migratory functions [9], upregulate integrin αvβ6, and respond to TGF-β1, by producing collagen type I. This feature is not observed in cultured normal cholangiocytes and may contribute to matrix deposition in the adjacent peribiliary area, where fibrogenesis starts (personal data). Here, PKHD1 is linked to congestive heart failure.