The oncometabolite 2-hydroxyglutarate activates the mTOR signalling pathway

Nat Commun. 2016 Sep 14:7:12700. doi: 10.1038/ncomms12700.

Abstract

The identification of cancer-associated mutations in the tricarboxylic acid (TCA) cycle enzymes isocitrate dehydrogenases 1 and 2 (IDH1/2) highlights the prevailing notion that aberrant metabolic function can contribute to carcinogenesis. IDH1/2 normally catalyse the oxidative decarboxylation of isocitrate into α-ketoglutarate (αKG). In gliomas and acute myeloid leukaemias, IDH1/2 mutations confer gain-of-function leading to production of the oncometabolite R-2-hydroxyglutarate (2HG) from αKG. Here we show that generation of 2HG by mutated IDH1/2 leads to the activation of mTOR by inhibiting KDM4A, an αKG-dependent enzyme of the Jumonji family of lysine demethylases. Furthermore, KDM4A associates with the DEP domain-containing mTOR-interacting protein (DEPTOR), a negative regulator of mTORC1/2. Depletion of KDM4A decreases DEPTOR protein stability. Our results provide an additional molecular mechanism for the oncogenic activity of mutant IDH1/2 by revealing an unprecedented link between TCA cycle defects and positive modulation of mTOR function downstream of the canonical PI3K/AKT/TSC1-2 pathway.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Astrocytes / metabolism
  • Citric Acid Cycle
  • Glioma / genetics
  • Glutarates / metabolism*
  • HEK293 Cells
  • HeLa Cells
  • Humans
  • Intracellular Signaling Peptides and Proteins / metabolism
  • Isocitrate Dehydrogenase / genetics*
  • Jumonji Domain-Containing Histone Demethylases / metabolism*
  • Mechanistic Target of Rapamycin Complex 1 / metabolism
  • Mechanistic Target of Rapamycin Complex 2 / metabolism
  • PTEN Phosphohydrolase / genetics
  • TOR Serine-Threonine Kinases / metabolism*
  • Ubiquitination
  • beta-Transducin Repeat-Containing Proteins / metabolism

Substances

  • BTRC protein, human
  • Glutarates
  • Intracellular Signaling Peptides and Proteins
  • beta-Transducin Repeat-Containing Proteins
  • alpha-hydroxyglutarate
  • IDH2 protein, human
  • Isocitrate Dehydrogenase
  • IDH1 protein, human
  • Jumonji Domain-Containing Histone Demethylases
  • KDM4A protein, human
  • DEPTOR protein, human
  • MTOR protein, human
  • Mechanistic Target of Rapamycin Complex 1
  • Mechanistic Target of Rapamycin Complex 2
  • TOR Serine-Threonine Kinases
  • PTEN Phosphohydrolase
  • PTEN protein, human