title: Prorenin receptor in kidney development.
abstract: Prorenin receptor (PRR), a receptor for renin and prorenin and an accessory subunit of the vacuolar proton pump H+-ATPase, is expressed in the developing kidney. Global loss of PRR is lethal in mice, and PRR mutations are associated with a high blood pressure, left ventricular hypertrophy and X-linked mental retardation in humans. With the advent of modern gene targeting techniques, including conditional knockout approaches, several recent studies have demonstrated critical roles for the PRR in several lineages of the developing kidney. PRR signaling has been shown to be essential for branching morphogenesis of the ureteric bud (UB), nephron progenitor survival and nephrogenesis. PRR regulates these developmental events through interactions with other transcription and growth factors. Several targeted PRR knockout animal models have structural defects mimicking congenital anomalies of the kidney and urinary tract observed in humans. The aim of this review, is to highlight new insights into the cellular and molecular mechanisms by which PRR may regulate UB branching, terminal differentiation and function of UB-derived collecting ducts, nephron progenitor maintenance, progression of nephrogenesis and normal structural kidney development and function.
authors.affiliation: Section of Pediatric Nephrology, Department of Pediatrics, Hypertension and Renal Center of Excellence, Tulane University Health Sciences Center, 1430 Tulane Avenue, SL-37, New Orleans, LA, 70112, USA. iiosipi@tulane.edu.
authors.forename: Ihor V
authors.initials: IV
authors.lastname: Yosypiv
mesh_terms: D000818:Animals; D002648:Child; D006801:Humans; D007668:Kidney / Q000254:growth & development* / Q000378:metabolism*; D007674:Kidney Diseases / Q000235:genetics / Q000378:metabolism; D051379:Mice; D011956:Receptors, Cell Surface / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction; D025262:Vacuolar Proton-Translocating ATPases / Q000235:genetics / Q000378:metabolism*; D000093342:Prorenin Receptor
chemical_list: C458319:ATP6AP2 protein, human; D011956:Receptors, Cell Surface; D025262:Vacuolar Proton-Translocating ATPases; D000093342:Prorenin Receptor
keywords: Kidney development; Nephron progenitors; Prorenin receptor; Renal hypodysplasia; Ureteric bud; Urine acidification