Pathological ribonuclease H1 causes R-loop depletion and aberrant DNA segregation in mitochondria

MPV17 Loss Causes Deoxynucleotide Insufficiency and Slow DNA Replication in Mitochondria

(2016) Ilaria Dalla Rosa et al.   PLoS Genetics

RNASEH1 Mutations Impair mtDNA Replication and Cause Adult-Onset Mitochondrial Encephalomyopathy

(2015) Aurelio Reyes et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Primer retention owing to the absence of RNase H1 is catastrophic for mitochondrial DNA replication

(2015) J. Bradley Holmes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Genetics and Disease

(2014) Estela Area-Gomez et al.   JOURNAL OF CHILD NEUROLOGY

MPV17L2 is required for ribosome assembly in mitochondria

(2014) Ilaria Dalla Rosa et al.   NUCLEIC ACIDS RESEARCH

Mutations in FBXL4 Cause Mitochondrial Encephalopathy and a Disorder of Mitochondrial DNA Maintenance

(2013) Penelope E. Bonnen et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutation of the human mitochondrial phenylalanine-tRNA synthetase causes infantile-onset epilepsy and cytochrome c oxidase deficiency

(2013) Abdulraheem Almalki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

MTERF1 Binds mtDNA to Prevent Transcriptional Interference at the Light-Strand Promoter but Is Dispensable for rRNA Gene Transcription Regulation

(2013) Mügen Terzioglu et al.   Cell Metabolism

Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease

(2013) Cornelia Kornblum et al.   NATURE GENETICS

Mitochondrial DNA replication proceeds via a ‘bootlace’ mechanism involving the incorporation of processed transcripts

(2013) Aurelio Reyes et al.   NUCLEIC ACIDS RESEARCH

Accessorizing the human mitochondrial transcription machinery

(2013) Megan L. Bestwick et al.   TRENDS IN BIOCHEMICAL SCIENCES

Human C4orf14 interacts with the mitochondrial nucleoid and is involved in the biogenesis of the small mitochondrial ribosomal subunit

(2012) J. He et al.   NUCLEIC ACIDS RESEARCH

Involvement of DNA ligase III and ribonuclease H1 in mitochondrial DNA replication in cultured human cells

(2011) Heini Ruhanen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Actin and myosin contribute to mammalian mitochondrial DNA maintenance

(2011) A. Reyes et al.   NUCLEIC ACIDS RESEARCH

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

(2011) C. Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mammalian Mitochondrial DNA Replication Intermediates Are Essentially Duplex but Contain Extensive Tracts of RNA/DNA Hybrid

(2010) Jaakko L.O. Pohjoismäki et al.   JOURNAL OF MOLECULAR BIOLOGY

The accessory subunit of mitochondrial DNA polymerase γ determines the DNA content of mitochondrial nucleoids in human cultured cells

(2009) M. Di Re et al.   NUCLEIC ACIDS RESEARCH