Notch in the kidney: development and disease

J Pathol. 2012 Jan;226(2):394-403. doi: 10.1002/path.2967. Epub 2011 Aug 24.


Notch signalling is a highly conserved cell-cell communication mechanism that regulates development, tissue homeostasis, and repair. Within the kidney, Notch has an important function in orchestrating kidney development. Recent studies indicate that Notch plays a key role in establishing proximal epithelial fate during nephron segmentation as well as the differentiation of principal cells in the renal collecting system. Notch signalling is markedly reduced in the adult kidney; however, increased Notch signalling has been noted in both acute and chronic kidney injury. Increased glomerular epithelial Notch signalling has been associated with albuminuria and glomerulosclerosis, while tubular epithelial Notch activation caused fibrosis development most likely inducing an improper epithelial repair pathway. Recent studies thereby indicate that Notch is a key regulator of kidney development, repair, and injury.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Carcinoma, Renal Cell / genetics
  • Carcinoma, Renal Cell / physiopathology
  • Carcinoma, Renal Cell / therapy
  • Cell Communication / physiology
  • Chronic Disease
  • Disease Models, Animal
  • Fibrosis / genetics
  • Fibrosis / pathology
  • Humans
  • Kidney / embryology*
  • Kidney / physiology
  • Kidney Diseases / genetics
  • Kidney Diseases / physiopathology*
  • Kidney Diseases / therapy
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / physiopathology
  • Kidney Neoplasms / therapy
  • Mutation / genetics
  • Receptors, Notch / antagonists & inhibitors
  • Receptors, Notch / genetics
  • Receptors, Notch / physiology*
  • Regeneration / physiology


  • Receptors, Notch