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The emergence of nephron progenitor cells (NPCs) in early embryonic life leads to the many rounds of nephrogenesis that result in a richly endowed kidney by the end of gestation. A delicate balance between NPC differentiation and self-renewal must be maintained to guarantee optimal nephron endowment. Genetic errors which disturb NPC cell fate can result in premature NPC depletion and renal hypoplasia/dysplasia or permit the β-catenin mutations that accompany malignant transformation into a Wilms tumor. Retention of a small population of NPCs scattered throughout the adult kidney are important for recovery from acute tubular injury later in life. In this review, we track the origin and characteristics of NPC, describe the phase of NPC priming prior to nephron induction and describe NPC differentiation during nephrogenesis. We then cover the role of NPC in human renal disease, including mechanisms by which quiescent NPCs repair the injured adult kidney and the human diseases linked to dysfunction of NPCs.