Notch Signaling in Nephron Segmentation

Joo Seop Park*, Raphael Kopan

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

3 Scopus citations

Abstract

Recent advances in our understanding of nephron segmentation highlight crucial roles of Notch signaling in this process. Differentiation of mesenchymal nephron progenitors is initiated by Wnt/β-catenin signaling. However, deactivation of Wnt/β-catenin signaling is required for both epithelialization of nephron progenitors and activation of Notch signaling. Studies on loss of function and gain of function showed that Notch signaling promotes differentiation of nephron progenitors into the proximal segment of the nephron while inhibiting the formation of the distal nephron segment (and, when overexpressed, of podocytes). Notch2 and Jag1 are the major receptor-ligand pair during nephrogenesis in the mouse model. Consistent with this, haploinsufficiency of either Jag1 or Notch2 in human causes Alagille syndrome, which includes kidney abnormalities.

Original languageEnglish (US)
Title of host publicationKidney Development, Disease, Repair and Regeneration
PublisherElsevier Science Ltd.
Pages87-93
Number of pages7
ISBN (Electronic)9780128004388
ISBN (Print)9780128001028
DOIs
StatePublished - Jan 1 2015

Keywords

  • Alagille syndrome
  • Nephrogenesis
  • Notch
  • Segmentation

ASJC Scopus subject areas

  • General Medicine

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