Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning

Hortensja Brzóska, Angela M. d'Esposito, Maria Kolatsi-Joannou, Vishal Patel, Peter Igarashi, Yunping Lei, Richard H. Finnell, Mark F. Lythgoe, Adrian S. Woolf, Eugenia Papakrivopoulou, David A. Long

Research output: Contribution to journalArticlepeer-review

19 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1274-1284
Number of pages11
JournalKidney international
Volume90
Issue number6
DOIs
StatePublished - Dec 1 2016

Keywords

  • kidney development
  • planar cell polarity
  • renal malformations
  • rostrocaudal patterning
  • ureteric branching

ASJC Scopus subject areas

  • Nephrology

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