Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNAHuman mitochondrial transcription revisited: only TFAM and TFB2M are required for transcription of the mitochondrial genes in vitroMTERF3 is a negative regulator of mammalian mtDNA transcriptionTWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding proteinThe mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cellsReconstitution of a minimal mtDNA replisome in vitroThe mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesisPrimer removal during mammalian mitochondrial DNA replicationMTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulationThe UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymesSelective mitochondrial DNA degradation following double-strand breaksCloning of the sea urchin mitochondrial RNA polymerase and reconstitution of the transcription termination systemUnraveling the structure of DNA during overstretching by using multicolor, single-molecule fluorescence imagingG-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation.Characterization of the mouse genes for mitochondrial transcription factors B1 and B2.MGME1 processes flaps into ligatable nicks in concert with DNA polymerase γ during mtDNA replication.Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA.Adenosine kinase deficiency disrupts the methionine cycle and causes hypermethioninemia, encephalopathy, and abnormal liver functionRole of human DNA glycosylase Nei-like 2 (NEIL2) and single strand break repair protein polynucleotide kinase 3'-phosphatase in maintenance of mitochondrial genome.Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid.Mammalian transcription factor A is a core component of the mitochondrial transcription machinery.Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies.DNA replication and transcription in mammalian mitochondria.TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replicationHuman mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro.The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria.Mitochondrial transcription termination factor 1 directs polar replication fork pausing.The human mitochondrial replication fork in health and disease.Maintenance and Expression of Mammalian Mitochondrial DNA.Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia.Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription.TEFM is a potent stimulator of mitochondrial transcription elongation in vitro.A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop.In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication.A family of putative transcription termination factors shared amongst metazoans and plants.The accessory subunit B of DNA polymerase gamma is required for mitochondrial replisome function.The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase activities.Structure-function defects of the twinkle amino-terminal region in progressive external ophthalmoplegia.Subnormal levels of POLγA cause inefficient initiation of light-strand DNA synthesis and lead to mitochondrial DNA deletions and progressive external ophthalmoplegia [corrected].Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA.
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P50
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