Cargando…
Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning
The mammalian kidney contains nephrons comprising glomeruli and tubules joined to ureteric bud–derived collecting ducts. It has a characteristic bean-like shape, with near-complete rostrocaudal symmetry around the hilum. Here we show that Celsr1, a planar cell polarity (PCP) gene implicated in neura...
Autores principales: | , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5126096/ https://www.ncbi.nlm.nih.gov/pubmed/27597235 http://dx.doi.org/10.1016/j.kint.2016.07.011 |
_version_ | 1782470057187606528 |
---|---|
author | Brzóska, Hortensja Ł. d’Esposito, Angela M. Kolatsi-Joannou, Maria Patel, Vishal Igarashi, Peter Lei, Yunping Finnell, Richard H. Lythgoe, Mark F. Woolf, Adrian S. Papakrivopoulou, Eugenia Long, David A. |
author_facet | Brzóska, Hortensja Ł. d’Esposito, Angela M. Kolatsi-Joannou, Maria Patel, Vishal Igarashi, Peter Lei, Yunping Finnell, Richard H. Lythgoe, Mark F. Woolf, Adrian S. Papakrivopoulou, Eugenia Long, David A. |
author_sort | Brzóska, Hortensja Ł. |
collection | PubMed |
description | The mammalian kidney contains nephrons comprising glomeruli and tubules joined to ureteric bud–derived collecting ducts. It has a characteristic bean-like shape, with near-complete rostrocaudal symmetry around the hilum. Here we show that Celsr1, a planar cell polarity (PCP) gene implicated in neural tube morphogenesis, is required for ureteric tree growth in early development and later in gestation prevents tubule overgrowth. We also found an interaction between Celsr1 and Vangl2 (another PCP gene) in ureteric tree growth, most marked in the caudal compartment of the kidneys from compound heterozygous mutant mice with a stunted rump. Furthermore, these genes together are required for the maturation of glomeruli. Interestingly, we demonstrated patients with CELSR1 mutations and spina bifida can have significant renal malformations. Thus, PCP genes are important in mammalian kidney development and have an unexpected role in rostrocaudal patterning during organogenesis. |
format | Online Article Text |
id | pubmed-5126096 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-51260962016-12-05 Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning Brzóska, Hortensja Ł. d’Esposito, Angela M. Kolatsi-Joannou, Maria Patel, Vishal Igarashi, Peter Lei, Yunping Finnell, Richard H. Lythgoe, Mark F. Woolf, Adrian S. Papakrivopoulou, Eugenia Long, David A. Kidney Int Basic Research The mammalian kidney contains nephrons comprising glomeruli and tubules joined to ureteric bud–derived collecting ducts. It has a characteristic bean-like shape, with near-complete rostrocaudal symmetry around the hilum. Here we show that Celsr1, a planar cell polarity (PCP) gene implicated in neural tube morphogenesis, is required for ureteric tree growth in early development and later in gestation prevents tubule overgrowth. We also found an interaction between Celsr1 and Vangl2 (another PCP gene) in ureteric tree growth, most marked in the caudal compartment of the kidneys from compound heterozygous mutant mice with a stunted rump. Furthermore, these genes together are required for the maturation of glomeruli. Interestingly, we demonstrated patients with CELSR1 mutations and spina bifida can have significant renal malformations. Thus, PCP genes are important in mammalian kidney development and have an unexpected role in rostrocaudal patterning during organogenesis. Elsevier 2016-12 /pmc/articles/PMC5126096/ /pubmed/27597235 http://dx.doi.org/10.1016/j.kint.2016.07.011 Text en © 2016 International Society of Nephrology. http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Basic Research Brzóska, Hortensja Ł. d’Esposito, Angela M. Kolatsi-Joannou, Maria Patel, Vishal Igarashi, Peter Lei, Yunping Finnell, Richard H. Lythgoe, Mark F. Woolf, Adrian S. Papakrivopoulou, Eugenia Long, David A. Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title | Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title_full | Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title_fullStr | Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title_full_unstemmed | Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title_short | Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
title_sort | planar cell polarity genes celsr1 and vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning |
topic | Basic Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5126096/ https://www.ncbi.nlm.nih.gov/pubmed/27597235 http://dx.doi.org/10.1016/j.kint.2016.07.011 |
work_keys_str_mv | AT brzoskahortensjał planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT despositoangelam planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT kolatsijoannoumaria planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT patelvishal planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT igarashipeter planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT leiyunping planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT finnellrichardh planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT lythgoemarkf planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT woolfadrians planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT papakrivopouloueugenia planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning AT longdavida planarcellpolaritygenescelsr1andvangl2arenecessaryforkidneygrowthdifferentiationandrostrocaudalpatterning |