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

Rachel L. Berry; Derya D. Ozdemir; Bruce Aronow; Nils O. Lindström; Tatiana Dudnakova; Anna Thornburn; Paul Perry; Richard Baldock; Chris Armit; Anagha Joshi; Cécile Jeanpierre; Jingdong Shan; Seppo Vainio; James Baily; David Brownstein; Jamie Davies; Nicholas D. Hastie; Peter Hohenstein

doi:10.1242/dmm.018523

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

Rachel L. Berry, Derya D. Ozdemir, Bruce Aronow, Nils O. Lindström, Tatiana Dudnakova, Anna Thornburn, Paul Perry, Richard Baldock, Chris Armit, Anagha Joshi, Cécile Jeanpierre, Jingdong Shan, Seppo Vainio, James Baily, David Brownstein, Jamie Davies, Nicholas D. Hastie, Peter Hohenstein^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

23 Scopus citations

Abstract

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 ofmouse 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.

Original language	English (US)
Pages (from-to)	903-917
Number of pages	15
Journal	DMM Disease Models and Mechanisms
Volume	8
Issue number	8
DOIs	https://doi.org/10.1242/dmm.018523
State	Published - Aug 1 2015
Externally published	Yes

Keywords

Kidney development
Mouse model
WT1
Wilms' tumour

ASJC Scopus subject areas

Neuroscience (miscellaneous)
Medicine (miscellaneous)
Immunology and Microbiology (miscellaneous)
General Biochemistry, Genetics and Molecular Biology

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1242/dmm.018523

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Berry, R. L., Ozdemir, D. D., Aronow, B., Lindström, N. O., Dudnakova, T., Thornburn, A., Perry, P., Baldock, R., Armit, C., Joshi, A., Jeanpierre, C., Shan, J., Vainio, S., Baily, J., Brownstein, D., Davies, J., Hastie, N. D., & Hohenstein, P. (2015). Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice. DMM Disease Models and Mechanisms, 8(8), 903-917. https://doi.org/10.1242/dmm.018523

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title = "Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice",

abstract = "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 ofmouse 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.",

keywords = "Kidney development, Mouse model, WT1, Wilms' tumour",

author = "Berry, {Rachel L.} and Ozdemir, {Derya D.} and Bruce Aronow and Lindstr{\"o}m, {Nils O.} and Tatiana Dudnakova and Anna Thornburn and Paul Perry and Richard Baldock and Chris Armit and Anagha Joshi and C{\'e}cile Jeanpierre and Jingdong Shan and Seppo Vainio and James Baily and David Brownstein and Jamie Davies and Hastie, {Nicholas D.} and Peter Hohenstein",

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doi = "10.1242/dmm.018523",

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Berry, RL, Ozdemir, DD, Aronow, B, Lindström, NO, Dudnakova, T, Thornburn, A, Perry, P, Baldock, R, Armit, C, Joshi, A, Jeanpierre, C, Shan, J, Vainio, S, Baily, J, Brownstein, D, Davies, J, Hastie, ND & Hohenstein, P 2015, 'Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice', DMM Disease Models and Mechanisms, vol. 8, no. 8, pp. 903-917. https://doi.org/10.1242/dmm.018523

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AU - Berry, Rachel L.

AU - Ozdemir, Derya D.

AU - Aronow, Bruce

AU - Lindström, Nils O.

AU - Dudnakova, Tatiana

AU - Thornburn, Anna

AU - Perry, Paul

AU - Baldock, Richard

AU - Armit, Chris

AU - Joshi, Anagha

AU - Jeanpierre, Cécile

AU - Shan, Jingdong

AU - Vainio, Seppo

AU - Baily, James

AU - Brownstein, David

AU - Davies, Jamie

AU - Hastie, Nicholas D.

AU - Hohenstein, Peter

PY - 2015/8/1

Y1 - 2015/8/1

N2 - 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 ofmouse 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.

AB - 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 ofmouse 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.

KW - Kidney development

KW - Mouse model

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

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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