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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...

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Autores principales: 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.
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
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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.
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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
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