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Single-stranded DNA (ssDNA) binding proteins protect regions of ssDNA formed during processes such as DNA replication and repair. We here devise a genetic screen and identify the mitochondrial ssDNA-binding protein (mtSSB) as a key regulator of mtDNA levels. In mitochondria, RNA synthesis from the l...
ORGANISM(S): Mus musculus (Mouse) 
2021-05-19 | PXD021799 | Pride
Abstract Dysfunction of the oxidative phosphorylation (OXPHOS) system is a major cause of human disease and the cellular consequences are highly complex. Here, we present comparative analyses of mitochondrial proteomes, cellular transcriptomes and targeted metabolomics of five knockout mouse strains...
ORGANISM(S): Mus musculus (Mouse) 
2022-08-09 | PXD035880 | Pride
Neurodegeneration in mitochondrial disorders is considered irreversible because of limited metabolic plasticity in neurons, yet the cell-autonomous implications of mitochondrial dysfunction for neuronal metabolism in vivo are poorly understood. Here, we profiled the cell-specific proteome of Purkinj...
ORGANISM(S): Mus musculus (Mouse) 
2020-08-03 | PXD019690 | Pride
Regulation of replication and expression of mitochondrial DNA (mtDNA) is essential for cellular energy conversion via oxidative phosphorylation. The mitochondrial transcription elongation factor (TEFM) has been proposed to regulate the switch between transcription termination for replication primer ...
ORGANISM(S): Homo sapiens (Human) Mus musculus (Mouse) 
2019-04-25 | PXD013398 | Pride
Mammalian mitochondrial DNA (mtDNA) is coated with mitochondrial transcription factor A (TFAM) and compacted into nucleoids. TFAM is not only the main component of mitochondrial nucleoids but its levels can also control mtDNA copy number. Here we show that the TFAM-to-mtDNA ratio is critical for mai...
ORGANISM(S): Mus musculus (Mouse) 
2021-08-18 | PXD023050 | Pride
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