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Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells.


ABSTRACT: Although the basic components and mechanisms of mitochondrial transcription in mammals have been described, the components involved in mRNA processing, translation and stability remain largely unknown. In plants, pentatricopeptide domain RNA-binding proteins regulate the stability, expression and translation of mitochondrial transcripts; therefore, we investigated the role of an uncharacterized mammalian pentatricopeptide domain protein, (PTCD1), in mitochondrial RNA metabolism. We show that PTCD1 is a mitochondrial matrix protein which associates with leucine tRNAs and precursor RNAs that contain leucine tRNAs. Knockdown of PTCD1 in 143B osteosarcoma cells did not change mitochondrial mRNA levels; however, it increased the abundance precursor RNAs and of leucine tRNAs and PTCD1 overexpression led to a reduction of these RNAs. Lowering PTCD1 in cells increased levels of several mitochondria-encoded proteins and Complex IV activity, suggesting that PTCD1 acts as a negative regulator of leucine tRNA levels and hence mitochondrial translation.

SUBMITTER: Rackham O 

PROVIDER: S-EPMC2761286 | biostudies-literature | 2009 Sep

REPOSITORIES: biostudies-literature

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Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells.

Rackham Oliver O   Davies Stefan M K SM   Shearwood Anne-Marie J AM   Hamilton Kristina L KL   Whelan James J   Filipovska Aleksandra A  

Nucleic acids research 20090803 17


Although the basic components and mechanisms of mitochondrial transcription in mammals have been described, the components involved in mRNA processing, translation and stability remain largely unknown. In plants, pentatricopeptide domain RNA-binding proteins regulate the stability, expression and translation of mitochondrial transcripts; therefore, we investigated the role of an uncharacterized mammalian pentatricopeptide domain protein, (PTCD1), in mitochondrial RNA metabolism. We show that PTC  ...[more]

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