The yin and yang of kidney development and Wilms' tumors.
Wilms' tumor, or nephroblastoma, is the most common pediatric renal cancer. The tumors morphologically resemble embryonic kidneys with a disrupted architecture and are associated with undifferentiated metanephric precursors. Here, we discuss genetic and epigenetic findings in Wilms' tumor in the context of renal development. Many of the genes implicated in Wilms' tumorigenesis are involved in the control of nephron progenitors or the microRNA (miRNA) processing pathway. Whereas the first group of genes has been extensively studied in normal development, the second finding suggests important roles for miRNAs in general-and specific miRNAs in particular-in normal kidney development that still await further analysis. The recent identification of Wilms' tumor cancer stem cells could provide a framework to integrate these pathways and translate them into new or improved therapeutic interventions.
The Roslin Institute, University of Edinburgh, Midlothian EH25 9RG, United Kingdom; MRC Human Genetics Unit, MRC Institute of Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom; peter.hohenstein@roslin.ed.ac.uk.
Peter
P
Hohenstein
UCL Institute of Child Health, University College London, London WC1N 1EH, United Kingdom.
Kathy
K
Pritchard-Jones
UCL Institute of Child Health, University College London, London WC1N 1EH, United Kingdom.
Jocelyn
J
Charlton
D000818:Animals; D044127:Epigenesis, Genetic / Q000235:genetics; D018507:Gene Expression Regulation, Developmental; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007668:Kidney / Q000196:embryology* / Q000473:pathology; D007680:Kidney Neoplasms / Q000235:genetics*; D035683:MicroRNAs / Q000235:genetics; D038081:Organogenesis / Q000235:genetics*; D009396:Wilms Tumor / Q000235:genetics*
D035683:MicroRNAs
Wilms’ tumor; Wt1; kidney development; miRNA; nephron progenitor cells; β-catenin