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PNPASE regulates RNA import into mitochondria.

RNA import into mammalian mitochondria is considered essential for replication, transcription, and translation of the mitochondrial genome but the pathway(s) and factors that control this import are poorly understood. Previously, we localized polynucleotide phosphorylase (PNPASE), a 3' --> 5' exoribonuclease and poly-A polymerase, in the mitochondrial intermembrane space, a location lacking resident RNAs. Here, we show a new role for PNPASE in regulating the import of nuclear-encoded RNAs into the mitochondrial matrix. PNPASE reduction impaired mitochondrial RNA processing and polycistronic transcripts accumulated. Augmented import of RNase P, 5S rRNA, and MRP RNAs depended on PNPASE expression and PNPASE-imported RNA interactions were identified. PNPASE RNA processing and import activities were separable and a mitochondrial RNA targeting signal was isolated that enabled RNA import in a PNPASE-dependent manner. Combined, these data strongly support an unanticipated role for PNPASE in mediating the translocation of RNAs into mitochondria.

Pubmed ID: 20691904


  • Wang G
  • Chen HW
  • Oktay Y
  • Zhang J
  • Allen EL
  • Smith GM
  • Fan KC
  • Hong JS
  • French SW
  • McCaffery JM
  • Lightowlers RN
  • Morse HC
  • Koehler CM
  • Teitell MA



Publication Data

August 6, 2010

Associated Grants

  • Agency: Wellcome Trust, Id: 074454
  • Agency: NCI NIH HHS, Id: K22CA120147
  • Agency: NEI NIH HHS, Id: PN2EY018228
  • Agency: NCI NIH HHS, Id: R01 CA090571
  • Agency: NCI NIH HHS, Id: R01 CA090571-09
  • Agency: NCI NIH HHS, Id: R01 CA107300
  • Agency: NCI NIH HHS, Id: R01 CA107300-05
  • Agency: NCI NIH HHS, Id: R01 CA156674
  • Agency: NIGMS NIH HHS, Id: R01 GM061721
  • Agency: NIGMS NIH HHS, Id: R01 GM073981
  • Agency: NCI NIH HHS, Id: R01CA90571
  • Agency: NIGMS NIH HHS, Id: R01GM061721
  • Agency: NIGMS NIH HHS, Id: R01GM073981
  • Agency: Intramural NIH HHS, Id:

Mesh Terms

  • Animals
  • Cell Line
  • Gene Knockout Techniques
  • Humans
  • Mice
  • Mice, Inbred C57BL
  • Mitochondria
  • Polyribonucleotide Nucleotidyltransferase
  • RNA
  • RNA Processing, Post-Transcriptional
  • Ribonuclease P
  • Saccharomyces cerevisiae