Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice.

Autor:	Berry RL; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Ozdemir DD; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK The Roslin Institute, University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK., Aronow B; Department of Biomedical Informatics and Developmental Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA., Lindström NO; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK The Roslin Institute, University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK., Dudnakova T; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Thornburn A; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Perry P; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Baldock R; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Armit C; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Joshi A; The Roslin Institute, University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK., Jeanpierre C; INSERM, UMR 1163, Laboratory of Inherited Kidney Diseases, Paris 75015, France Paris Descartes - Sorbonne Paris Cité University, Imagine Institute, Paris 75015, France., Shan J; Biocenter Oulu, InfoTech Oulu, Faculty of Biochemistry and Molecular Medicine, Aapistie 5A, University of Oulu, PO Box 5000, Oulu 90014, Finland., Vainio S; Biocenter Oulu, InfoTech Oulu, Faculty of Biochemistry and Molecular Medicine, Aapistie 5A, University of Oulu, PO Box 5000, Oulu 90014, Finland., Baily J; Queen's Medical Research Institute, University of Edinburgh, 47 Little France Crescent, Edinburgh, EH16 4TJ, UK., Brownstein D; Queen's Medical Research Institute, University of Edinburgh, 47 Little France Crescent, Edinburgh, EH16 4TJ, UK., Davies J; Centre for Integrative Physiology, University of Edinburgh, Hugh Robson Building, 15 George Square, Edinburgh, EH8 9XD, UK., Hastie ND; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK., Hohenstein P; MRC Human Genetics Unit, MRC Institute for Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, EH4 2XU, UK The Roslin Institute, University of Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, UK peter.hohenstein@roslin.ed.ac.uk.
Jazyk:	angličtina
Zdroj:	Disease models & mechanisms [Dis Model Mech] 2015 Aug 01; Vol. 8 (8), pp. 903-17. Date of Electronic Publication: 2015 May 14.
DOI:	10.1242/dmm.018523
Abstrakt:	Wilms' tumours, paediatric kidney cancers, are the archetypal example of tumours caused through the disruption of normal development. The genetically best-defined subgroup of Wilms' tumours is the group caused by biallelic loss of the WT1 tumour suppressor gene. Here, we describe a developmental series of mouse models with conditional loss of Wt1 in different stages of nephron development before and after the mesenchymal-to-epithelial transition (MET). We demonstrate that Wt1 is essential for normal development at all kidney developmental stages under study. Comparison of genome-wide expression data from the mutant mouse models with human tumour material of mutant or wild-type WT1 datasets identified the stage of origin of human WT1-mutant tumours, and emphasizes fundamental differences between the two human tumour groups due to different developmental stages of origin. (© 2015. Published by The Company of Biologists Ltd.)
Databáze:	MEDLINE
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