Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis

Maddalena Castelli, Manila Boca, Marco Chiaravalli, Harini Ramalingam, Isaline Rowe, Gianfranco Distefano, Thomas Carroll, Alessandra Boletta

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

Several organs, including the lungs and kidneys, are formed by epithelial tubes whose proper morphogenesis ensures correct function. This is best exemplified by the kidney, where defective establishment or maintenance of tubular diameter results in polycystic kidney disease, a common genetic disorder. Most polycystic kidney disease cases result from loss-of-function mutations in the PKD1 gene, encoding Polycystin-1, a large receptor of unknown function. Here we demonstrate that PC-1 has an essential role in the establishment of correct tubular diameter during nephron development. Polycystin-1 associates with Par3 favouring the assembly of a pro-polarizing Par3/aPKC complex and it regulates a programme of cell polarity important for oriented cell migration and for a convergent extension-like process during tubular morphogenesis. Par3 inactivation in the developing kidney results in defective convergent extension and tubular morphogenesis, and in renal cyst formation. Our data define Polycystin-1 as central to cell polarization and to epithelial tube morphogenesis and homeostasis.

Original languageEnglish (US)
Article number2658
JournalNature Communications
Volume4
DOIs
StatePublished - 2013

Fingerprint

kidneys
kidney diseases
Morphogenesis
Kidney
Polycystic Kidney Diseases
cells
tubes
homeostasis
cysts
Gene encoding
mutations
organs
genes
deactivation
lungs
Cell Polarity
Inborn Genetic Diseases
maintenance
Nephrons
polarity

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Chemistry(all)
  • Physics and Astronomy(all)

Cite this

Castelli, M., Boca, M., Chiaravalli, M., Ramalingam, H., Rowe, I., Distefano, G., ... Boletta, A. (2013). Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis. Nature Communications, 4, [2658]. https://doi.org/10.1038/ncomms3658

Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis. / Castelli, Maddalena; Boca, Manila; Chiaravalli, Marco; Ramalingam, Harini; Rowe, Isaline; Distefano, Gianfranco; Carroll, Thomas; Boletta, Alessandra.

In: Nature Communications, Vol. 4, 2658, 2013.

Research output: Contribution to journalArticle

Castelli, M, Boca, M, Chiaravalli, M, Ramalingam, H, Rowe, I, Distefano, G, Carroll, T & Boletta, A 2013, 'Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis', Nature Communications, vol. 4, 2658. https://doi.org/10.1038/ncomms3658
Castelli, Maddalena ; Boca, Manila ; Chiaravalli, Marco ; Ramalingam, Harini ; Rowe, Isaline ; Distefano, Gianfranco ; Carroll, Thomas ; Boletta, Alessandra. / Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis. In: Nature Communications. 2013 ; Vol. 4.
@article{24000d52d1734e8f978929c005d6158b,
title = "Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis",
abstract = "Several organs, including the lungs and kidneys, are formed by epithelial tubes whose proper morphogenesis ensures correct function. This is best exemplified by the kidney, where defective establishment or maintenance of tubular diameter results in polycystic kidney disease, a common genetic disorder. Most polycystic kidney disease cases result from loss-of-function mutations in the PKD1 gene, encoding Polycystin-1, a large receptor of unknown function. Here we demonstrate that PC-1 has an essential role in the establishment of correct tubular diameter during nephron development. Polycystin-1 associates with Par3 favouring the assembly of a pro-polarizing Par3/aPKC complex and it regulates a programme of cell polarity important for oriented cell migration and for a convergent extension-like process during tubular morphogenesis. Par3 inactivation in the developing kidney results in defective convergent extension and tubular morphogenesis, and in renal cyst formation. Our data define Polycystin-1 as central to cell polarization and to epithelial tube morphogenesis and homeostasis.",
author = "Maddalena Castelli and Manila Boca and Marco Chiaravalli and Harini Ramalingam and Isaline Rowe and Gianfranco Distefano and Thomas Carroll and Alessandra Boletta",
year = "2013",
doi = "10.1038/ncomms3658",
language = "English (US)",
volume = "4",
journal = "Nature Communications",
issn = "2041-1723",
publisher = "Nature Publishing Group",

}

TY - JOUR

T1 - Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis

AU - Castelli, Maddalena

AU - Boca, Manila

AU - Chiaravalli, Marco

AU - Ramalingam, Harini

AU - Rowe, Isaline

AU - Distefano, Gianfranco

AU - Carroll, Thomas

AU - Boletta, Alessandra

PY - 2013

Y1 - 2013

N2 - Several organs, including the lungs and kidneys, are formed by epithelial tubes whose proper morphogenesis ensures correct function. This is best exemplified by the kidney, where defective establishment or maintenance of tubular diameter results in polycystic kidney disease, a common genetic disorder. Most polycystic kidney disease cases result from loss-of-function mutations in the PKD1 gene, encoding Polycystin-1, a large receptor of unknown function. Here we demonstrate that PC-1 has an essential role in the establishment of correct tubular diameter during nephron development. Polycystin-1 associates with Par3 favouring the assembly of a pro-polarizing Par3/aPKC complex and it regulates a programme of cell polarity important for oriented cell migration and for a convergent extension-like process during tubular morphogenesis. Par3 inactivation in the developing kidney results in defective convergent extension and tubular morphogenesis, and in renal cyst formation. Our data define Polycystin-1 as central to cell polarization and to epithelial tube morphogenesis and homeostasis.

AB - Several organs, including the lungs and kidneys, are formed by epithelial tubes whose proper morphogenesis ensures correct function. This is best exemplified by the kidney, where defective establishment or maintenance of tubular diameter results in polycystic kidney disease, a common genetic disorder. Most polycystic kidney disease cases result from loss-of-function mutations in the PKD1 gene, encoding Polycystin-1, a large receptor of unknown function. Here we demonstrate that PC-1 has an essential role in the establishment of correct tubular diameter during nephron development. Polycystin-1 associates with Par3 favouring the assembly of a pro-polarizing Par3/aPKC complex and it regulates a programme of cell polarity important for oriented cell migration and for a convergent extension-like process during tubular morphogenesis. Par3 inactivation in the developing kidney results in defective convergent extension and tubular morphogenesis, and in renal cyst formation. Our data define Polycystin-1 as central to cell polarization and to epithelial tube morphogenesis and homeostasis.

UR - http://www.scopus.com/inward/record.url?scp=84886654516&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84886654516&partnerID=8YFLogxK

U2 - 10.1038/ncomms3658

DO - 10.1038/ncomms3658

M3 - Article

C2 - 24153433

AN - SCOPUS:84886654516

VL - 4

JO - Nature Communications

JF - Nature Communications

SN - 2041-1723

M1 - 2658

ER -