NDPK-D (NM23-H4)-mediated externalization of cardiolipin enables elimination of depolarized mitochondria by mitophagy

Cell Death Differ. 2016 Jul;23(7):1140-51. doi: 10.1038/cdd.2015.160. Epub 2016 Jan 8.

Abstract

Mitophagy is critical for cell homeostasis. Externalization of the inner mitochondrial membrane phospholipid, cardiolipin (CL), to the surface of the outer mitochondrial membrane (OMM) was identified as a mitophageal signal recognized by the microtubule-associated protein 1 light chain 3. However, the CL-translocating machinery remains unknown. Here we demonstrate that a hexameric intermembrane space protein, NDPK-D (or NM23-H4), binds CL and facilitates its redistribution to the OMM. We found that mitophagy induced by a protonophoric uncoupler, carbonyl cyanide m-chlorophenylhydrazone (CCCP), caused externalization of CL to the surface of mitochondria in murine lung epithelial MLE-12 cells and human cervical adenocarcinoma HeLa cells. RNAi knockdown of endogenous NDPK-D decreased CCCP-induced CL externalization and mitochondrial degradation. A R90D NDPK-D mutant that does not bind CL was inactive in promoting mitophagy. Similarly, rotenone and 6-hydroxydopamine triggered mitophagy in SH-SY5Y cells was also suppressed by knocking down of NDPK-D. In situ proximity ligation assay (PLA) showed that mitophagy-inducing CL-transfer activity of NDPK-D is closely associated with the dynamin-like GTPase OPA1, implicating fission-fusion dynamics in mitophagy regulation.

MeSH terms

  • Animals
  • Autophagy / drug effects
  • Carbonyl Cyanide m-Chlorophenyl Hydrazone / toxicity
  • Cardiolipins / analysis
  • Cardiolipins / metabolism*
  • Cell Line
  • GTP Phosphohydrolases / metabolism
  • HeLa Cells
  • Humans
  • Lysosomes / metabolism
  • Lysosomes / pathology
  • Mice
  • Microtubule-Associated Proteins / metabolism
  • Mitochondria / metabolism*
  • Mitochondria / pathology
  • Mitochondrial Membranes / metabolism*
  • Mitophagy* / drug effects
  • Mutagenesis, Site-Directed
  • Nucleoside Diphosphate Kinase D / antagonists & inhibitors
  • Nucleoside Diphosphate Kinase D / genetics
  • Nucleoside Diphosphate Kinase D / metabolism*
  • Oxidopamine / pharmacology
  • Protein Binding
  • RNA Interference
  • Rotenone / pharmacology

Substances

  • Cardiolipins
  • Microtubule-Associated Proteins
  • Rotenone
  • Carbonyl Cyanide m-Chlorophenyl Hydrazone
  • Oxidopamine
  • NME4 protein, human
  • Nucleoside Diphosphate Kinase D
  • GTP Phosphohydrolases
  • OPA1 protein, human