Replication and expression of the mitochondrial genome depend on the sufficient supply of
nucleotide building blocks to mitochondria. Dysregulated nucleotide metabolism is detrimental to
mitochondrial genomes and can destabilise mitochondrial DNA and trigger inflammation. Here, we
report that a mitochondrial nucleoside diphosphate kinase, NME6, supplies mitochondria with
pyrimidine ribonucleotides to drive the transcription of mitochondrial genes. Perturbation of NME6
leads to the depletion of mitochondrial transcripts, destabilisation of the electron transport chain
and impaired oxidative phosphorylation. These deficiencies are rescued upon supplementation with
pyrimidine ribonucleosides. Moreover, NME6 is required for the maintenance of mitochondrial DNA
when the access to cytosolic pyrimidine deoxyribonucleotides is limited. Our results, therefore, shed
light on the importance of mitochondrial ribonucleotide salvage for the synthesis of RNA and
mitochondrial gene expression.
The repository contains two internal projects which are identified by the internal IDs: 0187 and 0196. The raw files are indicated by the internal ID, and each Supplementary Table contains a reference to the internal ID. The project with the ID 0187 contains whole cell (WC) and immunoprecipitation (IP) data. Please see the file: SampleNamesForProjects.xlsx for more details.