Baggio 2014 Nucleic Acids Res

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Publications in the MiPMap
Baggio F, Bratic A, Mourier A, Kauppila TE, Tain LS, Kukat C, Habermann B, Partridge L, Larsson NG5 (2014) Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation. Nucleic Acids Res 42:13920-38.

» PMID: 25428350

Baggio F, Bratic A, Mourier A, Kauppila TE, Tain LS, Kukat C, Habermann B, Partridge L, Larsson NG5 (2014) Nucleic Acids Res

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

Keywords: DmLrpprc2, Leigh syndrome

O2k-Network Lab: DE Cologne Larsson NG


Labels: MiParea: Respiration, mtDNA;mt-genetics, Comparative MiP;environmental MiP 


Organism: Drosophila, Hexapods 

Preparation: Permeabilized tissue 


Coupling state: LEAK, OXPHOS, ET  Pathway: N, S, Gp, CIV, Other combinations  HRR: Oxygraph-2k