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

Maddalena Castelli1, Manila Boca, Marco Chiaravalli

  • 11] Division of Genetics and Cell Biology, Dulbecco Telethon Institute (DTI) at Dibit, San Raffaele Scientific Institute, Via Olgettina, 58, 20132 Milan, Italy [2] Ph.D. Program in Molecular and Cellular Biology, Vita-Salute San Raffaele University, Via Olgettina, 58, 20132 Milan, Italy.

Nature Communications
|October 25, 2013
PubMed

Insights

Polycystin-1 (PC-1) is crucial for kidney tubule diameter during development. It regulates cell polarity, impacting kidney morphogenesis and preventing polycystic kidney disease.

Area of Science:

  • Developmental Biology
  • Cell Biology
  • Genetics

Background:

  • Epithelial tubes, like those in the kidneys and lungs, require proper morphogenesis for function.
  • Polycystic kidney disease (PKD) arises from defects in tubular diameter, often due to mutations in the PKD1 gene.
  • The function of Polycystin-1 (PC-1), encoded by PKD1, remains largely unknown.

Purpose of the Study:

  • To elucidate the role of Polycystin-1 (PC-1) in kidney development and tubular morphogenesis.
  • To investigate the molecular mechanisms by which PC-1 influences epithelial tube formation.
  • To understand the link between PC-1 function, cell polarity, and the pathogenesis of PKD.

Main Methods:

  • Investigated the association of PC-1 with cell polarity proteins.
  • Analyzed the effects of PC-1 and Par3 manipulation on kidney tubular development in vivo.
  • Utilized genetic models to study the impact of gene inactivation on renal morphogenesis.

Main Results:

  • PC-1 directly associates with Par3, promoting the formation of a pro-polarizing Par3/aPKC complex.
  • PC-1 regulates cell polarity, essential for oriented cell migration and convergent extension during kidney tubule development.
  • Inactivation of Par3 in developing kidneys led to impaired convergent extension, abnormal tubular morphogenesis, and cyst formation.

Conclusions:

  • Polycystin-1 (PC-1) plays a critical role in establishing correct tubular diameter during nephron development.
  • PC-1 is central to regulating cell polarization, a key process for epithelial tube morphogenesis.
  • Dysregulation of PC-1 mediated cell polarity contributes to the development of polycystic kidney disease.

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