Mammalian mitochondrial DNA replication and mechanisms of deletion formation

TEFM regulates both transcription elongation and RNA processing in mitochondria

(2019) Shan Jiang et al.   EMBO REPORTS

Organization of DNA in Mammalian Mitochondria

(2019) Géraldine Farge et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondrial single-stranded DNA binding protein novel de novo SSBP1 mutation in a child with single large-scale mtDNA deletion (SLSMD) clinically manifesting as Pearson, Kearns-Sayre, and Leigh syndromes

(2019) Margaret A. Gustafson et al.   PLoS One

SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder

(2019) Valentina Del Dotto et al.   JOURNAL OF CLINICAL INVESTIGATION

Structural basis of mitochondrial transcription

(2018) Hauke S. Hillen et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A two-nuclease pathway involving RNase H1 is required for primer removal at human mitochondrial OriL

(2018) Ali Al-Behadili et al.   NUCLEIC ACIDS RESEARCH

Single-molecule DREEM imaging reveals DNA wrapping around human mitochondrial single-stranded DNA binding protein

(2018) Parminder Kaur et al.   NUCLEIC ACIDS RESEARCH

DNA polymerases in the mitochondria A critical review of the evidence

(2017) William C Copeland   Frontiers in Bioscience-Landmark

Transcription profiling suggests that mitochondrial topoisomerase IB acts as a topological barrier and regulator of mitochondrial DNA transcription

(2017) Ilaria Dalla Rosa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

Regulation of DNA replication at the end of the mitochondrial D-loop involves the helicase TWINKLE and a conserved sequence element

(2015) Elisabeth Jemt et al.   NUCLEIC ACIDS RESEARCH

Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid

(2015) Christian Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Replication-transcription switch in human mitochondria

(2015) K. Agaronyan et al.   SCIENCE

Unique features of DNA replication in mitochondria: A functional and evolutionary perspective

(2014) Ian J. Holt et al.   BIOESSAYS

In Vitro-Reconstituted Nucleoids Can Block Mitochondrial DNA Replication and Transcription

(2014) Géraldine Farge et al.   Cell Reports

PrimPol, an Archaic Primase/Polymerase Operating in Human Cells

(2013) Sara García-Gómez et al.   MOLECULAR CELL

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

(2013) Cornelia Kornblum et al.   NATURE GENETICS

Polg2 is essential for mammalian embryogenesis and is required for mtDNA maintenance

(2012) Margaret M. Humble et al.   HUMAN MOLECULAR GENETICS

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

Mitochondrial Topoisomerase I is Critical for Mitochondrial Integrity and Cellular Energy Metabolism

(2012) Céline Douarre et al.   PLoS One

Protein sliding and DNA denaturation are essential for DNA organization by human mitochondrial transcription factor A

(2012) Géraldine Farge et al.   Nature Communications

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

Mitochondrial RNA Polymerase Is Needed for Activation of the Origin of Light-Strand DNA Replication

(2010) Javier Miralles Fusté et al.   MOLECULAR CELL

Ribonuclease H: the enzymes in eukaryotes

(2009) Susana M. Cerritelli et al.   FEBS Journal

Mice expressing an error-prone DNA polymerase in mitochondria display elevated replication pausing and chromosomal breakage at fragile sites of mitochondrial DNA

(2009) Laura J. Bailey et al.   NUCLEIC ACIDS RESEARCH

Structure–Function Defects of the TWINKLE Linker Region in Progressive External Ophthalmoplegia

(2008) Jenny A. Korhonen et al.   JOURNAL OF MOLECULAR BIOLOGY