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Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease
It is shown that MGME1-mediated mtDNA processing is essential for mitochondrial genome maintenance. Expand
TEFM (c17orf42) is necessary for transcription of human mtDNA
It is shown that c17orf42, hereafter TEFM (transcription elongation factor of mitochondria), makes a critical contribution to mitochondrial transcription, and it is proposed that TEFM is a mitochondrial transcription elongation regulator. Expand
MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome
Inactivation of MRM2 or MRM3 in human cells by RNAi results in respiratory incompetence owing to diminished mitochondrial translation and the aberrant assembly of the large subunit of the mitochondrial ribosome. Expand
Chimeric DNA methyltransferases target DNA methylation to specific DNA sequences and repress expression of target genes
It is shown that it is possible to direct DNA MTase activity to predetermined sites in DNA, achieve targeted gene silencing in mammalian cell lines and interfere with HSV-1 propagation. Expand
PDE12 removes mitochondrial RNA poly(A) tails and controls translation in human mitochondria
Polyadenylation of mRNA in human mitochondria is crucial for gene expression and perturbation of poly(A) tail length has been linked to a human neurodegenerative disease. Here we show thatExpand
The Yeast Mitochondrial Degradosome
The purified degradosome has RNA helicase activity that precedes and is essential for exoribonuclease activity of this complex and is suggested to be a central part of a mitochondrial RNA surveillance system responsible for degradation of aberrant and unprocessed RNAs. Expand
Deficient methylation and formylation of mt-tRNAMet wobble cytosine in a patient carrying mutations in NSUN3
It is shown that NSun3 is required for deposition of m5C at the anticodon loop in the mitochondrially encoded transfer RNA methionine (mt-tRNAMet), and f5C in human mitochondrial RNA is generated by oxidative processing of m 5C. Expand
Localisation of the human hSuv3p helicase in the mitochondrial matrix and its preferential unwinding of dsDNA.
The presented analysis of the hSuv3p NTPase/helicase suggests that new functions of the protein have been acquired in the course of evolution. Expand
Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances
The latest breakthroughs in understanding of the post-transcriptional processes of mitochondrial gene expression are reviewed, focusing on advances in analyzing the mitochondrial epitranscriptome, the role of mitochondrial RNA granules (MRGs), the benefits of recently obtained structures of the mitochondrial ribosome, and the coordination of mitochondrial and cytosolic translation to orchestrate the biogenesis of OxPhos complexes. Expand
Mitochondrial transcription and translation: overview
The expression of mtDNA is vital for the assembly and functioning of the oxidative phosphorylation complexes, and defects of the mechanisms regulating mtDNA gene expression have been associated with deficiencies in assembly of these complexes, resulting in mitochondrial diseases. Expand