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Abstract
Autosomal dominant polycystic kidney disease is the most prevalent, potentially lethal
monogenic disorder. It has large inter- and intra-familial variability explained to
a large extent by its genetic heterogeneity and modifier genes. An increased understanding
of its underlying genetic, molecular, and cellular mechanisms and a better appreciation
of its progression and systemic manifestations have laid out the foundation for the
development of clinical trials and potentially effective therapies. The purpose of
this review is to update the core of knowledge in this area with recent publications
that have appeared during 2006-2009.