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- Baggio 2014 Nucleic Acids Res + (Members of the pentatricopeptide repeat do … Members of the pentatricopeptide repeat domain</br>(PPR) protein family bind RNA and are important</br>for post-transcriptional control of organelle gene expression</br>in unicellular eukaryotes, metazoans and</br>plants. They also have a role in human pathology,</br>as mutations in the leucine-rich PPR-containing</br>(LRPPRC) gene cause severe neurodegeneration.We</br>have previously shown that the mammalian LRPPRC</br>protein and its ''Drosophila melanogaster'' homolog</br>DmLRPPRC1 (also known as bicoid stability factor)</br>are necessary for mitochondrial translation by controlling</br>stability and polyadenylation of mRNAs. We</br>here report characterization of DmLRPPRC2, a second</br>fruit fly homolog of LRPPRC, and show that it</br>has a predominant mitochondrial localization and</br>interacts with a stem-loop interacting RNA binding</br>protein (DmSLIRP2). Ubiquitous downregulation of</br>DmLrpprc2 expression causes respiratory chain dysfunction,</br>developmental delay and shortened lifespan.</br>Unexpectedly, decreased DmLRPPRC2 expression</br>does not globally affect steady-state levels or</br>polyadenylation of mitochondrial transcripts. However,</br>some mitochondrial transcripts abnormally associate</br>with the mitochondrial ribosomes and some</br>products are dramatically overproduced and other</br>ones decreased, which, in turn, results in severe deficiency</br>of respiratory chain complexes. The function</br>of DmLRPPRC2 thus seems to be to ensure that</br>mitochondrial transcripts are presented to the mitochondrial</br>ribosomes in an orderly fashion to avoid</br>poorly coordinated translation.n to avoid poorly coordinated translation.)
