Standard texts describe human autosomal recessive polycystic kidney disease (ARPKD) as
a cystic kidney disease in which lesions are localized to collecting tubules. Murine models of
ARPKD consistently demonstrate an early phase of proximal tubular (PT) cystic involvement,
which disappears shortly after birth. This is followed by a phase of collecting tubular (CT)
cyst formation and progressive enlargement leading to compromise of renal function and
death. Because the description of cystic lesions in human ARPKD has been largely based …