1-Deoxydihydroceramide causes anoxic death by impairing chaperonin-mediated protein folding

Nat Metab. 2019 Oct;1(10):996-1008. doi: 10.1038/s42255-019-0123-y. Epub 2019 Oct 14.

Abstract

Ischaemic heart disease and stroke are the most common causes of death worldwide. Anoxia, defined as the lack of oxygen, is commonly seen in both these pathologies and triggers profound metabolic and cellular changes. Sphingolipids have been implicated in anoxia injury, but the pathomechanism is unknown. Here we show that anoxia-associated injury causes accumulation of the non-canonical sphingolipid 1-deoxydihydroceramide (DoxDHCer). Anoxia causes an imbalance between serine and alanine resulting in a switch from normal serine-derived sphinganine biosynthesis to non-canonical alanine-derived 1-deoxysphinganine. 1-Deoxysphinganine is incorporated into DoxDHCer, which impairs actin folding via the cytosolic chaperonin TRiC, leading to growth arrest in yeast, increased cell death upon anoxia-reoxygenation in worms and ischaemia-reperfusion injury in mouse hearts. Prevention of DoxDHCer accumulation in worms and in mouse hearts resulted in decreased anoxia-induced injury. These findings unravel key metabolic changes during oxygen deprivation and point to novel strategies to avoid tissue damage and death.

Publication types

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

MeSH terms

  • Alanine / metabolism
  • Animals
  • Animals, Genetically Modified
  • Caenorhabditis elegans
  • Cell Division
  • Chaperonins / genetics
  • Chaperonins / metabolism*
  • Feeding Behavior
  • Hypoxia / chemically induced*
  • Ion Channels / metabolism
  • Mice
  • Mice, Inbred C57BL
  • Mutation
  • Myocardial Reperfusion Injury / prevention & control
  • Protein Folding / drug effects*
  • Saccharomyces cerevisiae / metabolism
  • Serine / metabolism
  • Sphingosine / analogs & derivatives
  • Sphingosine / metabolism

Substances

  • Ion Channels
  • TRIC-A protein, mouse
  • Serine
  • Chaperonins
  • Sphingosine
  • Alanine
  • safingol