Hexokinase 1 forms rings that regulate mitochondrial fission during energy stress

Mol Cell. 2024 Jul 25;84(14):2732-2746.e5. doi: 10.1016/j.molcel.2024.06.009. Epub 2024 Jul 8.

Abstract

Metabolic enzymes can adapt during energy stress, but the consequences of these adaptations remain understudied. Here, we discovered that hexokinase 1 (HK1), a key glycolytic enzyme, forms rings around mitochondria during energy stress. These HK1-rings constrict mitochondria at contact sites with the endoplasmic reticulum (ER) and mitochondrial dynamics protein (MiD51). HK1-rings prevent mitochondrial fission by displacing the dynamin-related protein 1 (Drp1) from mitochondrial fission factor (Mff) and mitochondrial fission 1 protein (Fis1). The disassembly of HK1-rings during energy restoration correlated with mitochondrial fission. Mechanistically, we identified that the lack of ATP and glucose-6-phosphate (G6P) promotes the formation of HK1-rings. Mutations that affect the formation of HK1-rings showed that HK1-rings rewire cellular metabolism toward increased TCA cycle activity. Our findings highlight that HK1 is an energy stress sensor that regulates the shape, connectivity, and metabolic activity of mitochondria. Thus, the formation of HK1-rings may affect mitochondrial function in energy-stress-related pathologies.

Keywords: ER-mitochondria contact sites; energy stress; glucose starvation; glycolysis; hexokinase; live-cell imaging; mitochondrial constriction; mitochondrial fission; non-catalytic functions; protein cluster.

MeSH terms

  • Adenosine Triphosphate / metabolism
  • Animals
  • Citric Acid Cycle
  • Dynamins* / genetics
  • Dynamins* / metabolism
  • Endoplasmic Reticulum / metabolism
  • Energy Metabolism*
  • GTP Phosphohydrolases / genetics
  • GTP Phosphohydrolases / metabolism
  • Glucose-6-Phosphate / metabolism
  • HEK293 Cells
  • HeLa Cells
  • Hexokinase* / genetics
  • Hexokinase* / metabolism
  • Humans
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Mice
  • Mitochondria* / enzymology
  • Mitochondria* / genetics
  • Mitochondria* / metabolism
  • Mitochondrial Dynamics*
  • Mitochondrial Proteins* / genetics
  • Mitochondrial Proteins* / metabolism
  • Mutation
  • Stress, Physiological

Substances

  • Hexokinase
  • HK1 protein, human
  • Dynamins
  • Mitochondrial Proteins
  • Adenosine Triphosphate
  • DNM1L protein, human
  • Membrane Proteins
  • Glucose-6-Phosphate
  • Mff protein, human
  • GTP Phosphohydrolases