Nrf2 controls iron homeostasis in haemochromatosis and thalassaemia via Bmp6 and hepcidin

Nat Metab. 2019 May;1(5):519-531. doi: 10.1038/s42255-019-0063-6. Epub 2019 May 13.

Abstract

Iron is critical for life but toxic in excess because of iron-catalysed formation of pro-oxidants that cause tissue damage in a range of disorders. The Nrf2 transcription factor orchestrates cell-intrinsic protective antioxidant responses, and the peptide hormone hepcidin maintains systemic iron homeostasis, but is pathophysiologically decreased in haemochromatosis and beta-thalassaemia. Here, we show that Nrf2 is activated by iron-induced, mitochondria-derived pro-oxidants and drives Bmp6 expression in liver sinusoid endothelial cells, which in turn increases hepcidin synthesis by neighbouring hepatocytes. In Nrf2 knockout mice, the Bmp6-hepcidin response to oral and parenteral iron is impaired and iron accumulation and hepatic damage are increased. Pharmacological activation of Nrf2 stimulates the Bmp6-hepcidin axis, improving iron homeostasis in haemochromatosis and counteracting the inhibition of Bmp6 by erythroferrone in beta-thalassaemia. We propose that Nrf2 links cellular sensing of excess toxic iron to control of systemic iron homeostasis and antioxidant responses, and may be a therapeutic target for iron-associated disorders.

Publication types

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

MeSH terms

  • Bone Morphogenetic Protein 6 / physiology*
  • Hepcidins / physiology*
  • Homeostasis / physiology*
  • Humans
  • Iron / metabolism*
  • NF-E2-Related Factor 2 / physiology*
  • beta-Thalassemia / physiopathology*

Substances

  • BMP6 protein, human
  • Bone Morphogenetic Protein 6
  • Hepcidins
  • NF-E2-Related Factor 2
  • NFE2L2 protein, human
  • Iron