List of works - Maria Falkenberg

A family of putative transcription termination factors shared amongst metazoans and plants

scientific article published on October 2005

A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop

scientific article published on 10 September 2012

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

Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing

scientific article published on 14 December 2020

Adenosine kinase deficiency disrupts the methionine cycle and causes hypermethioninemia, encephalopathy, and abnormal liver function

scientific article

Characterization of the mouse genes for mitochondrial transcription factors B1 and B2.

scientific article

Cloning of the sea urchin mitochondrial RNA polymerase and reconstitution of the transcription termination system

scientific article

Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies

scientific article published on 10 November 2015

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

scientific article published on 24 August 2015

DNA replication and transcription in mammalian mitochondria

scientific article published on January 2007

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

scientific article published on 6 March 2018

G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation

scientific article

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

scholarly article

Human mitochondrial transcription revisited: only TFAM and TFB2M are required for transcription of the mitochondrial genes in vitro

scientific article

In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication

scientific article published on 23 October 2012

MGME1 processes flaps into ligatable nicks in concert with DNA polymerase γ during mtDNA replication

scientific article

MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation

scientific journal article

MTERF3 is a negative regulator of mammalian mtDNA transcription

scientific article

Maintenance and Expression of Mammalian Mitochondrial DNA.

scientific article published on 24 March 2016

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription

scientific article published on 20 June 2010

Mammalian mitochondrial DNA replication and mechanisms of deletion formation

scientific article published on 24 September 2020

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery

scientific article

Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria.

scientific article published on 23 March 2018

Mitochondrial DNA replication in mammalian cells: overview of the pathway

scholarly article by Maria Falkenberg published in July 2018

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

scientific article published on 11 March 2004

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

scientific article

Mitochondrial transcription termination factor 1 directs polar replication fork pausing

scientific article published on 25 April 2016

POLRMT mutations impair mitochondrial transcription causing neurological disease

scientific article published on 18 February 2021

Primer removal during mammalian mitochondrial DNA replication

scientific article (publication date: October 2015)

RNase H1 directs origin-specific initiation of DNA replication in human mitochondria

article published in 2019

Reconstitution of a minimal mtDNA replisome in vitro

scientific article

Ribonucleotides embedded in template DNA impair mitochondrial RNA polymerase progression

Role of human DNA glycosylase Nei-like 2 (NEIL2) and single strand break repair protein polynucleotide kinase 3'-phosphatase in maintenance of mitochondrial genome

scientific article published on 30 November 2011

Selective mitochondrial DNA degradation following double-strand breaks

scientific article

Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia

scientific article published on 12 January 2011

Small-molecule inhibitors of human mitochondrial DNA transcription

scientific article published on 04 January 2021

Structural basis for adPEO-causing mutations in the mitochondrial TWINKLE helicase

scientific article published on 01 April 2019

Structure-function defects of the TWINKLE linker region in progressive external ophthalmoplegia

scientific article published on 26 January 2008

Structure-function defects of the twinkle amino-terminal region in progressive external ophthalmoplegia

scientific article published on 24 November 2008

Subnormal levels of POLγA cause inefficient initiation of light-strand DNA synthesis and lead to mitochondrial DNA deletions and progressive external ophthalmoplegia [corrected].

scientific article published on 27 February 2013

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

scientific article

TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

scientific article published on 17 February 2015

TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein

scientific article

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication

scientific article published on 7 February 2013

The DNA Ligands Influence the Interactions between the Herpes Simplex Virus 1 Origin Binding Protein and the Single Strand DNA-binding Protein, ICP-8 ⬇️

scientific article published on August 11, 1995

The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase activities

scientific article published on 26 November 2007

The UL8 Subunit of the Heterotrimeric Herpes Simplex Virus Type 1 Helicase-Primase Is Required for the Unwinding of Single Strand DNA-binding Protein (ICP8)-coated DNA Substrates ⬇️

scientific article published on September 5, 1997

The UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymes

scientific journal article

The accessory subunit B of DNA polymerase gamma is required for mitochondrial replisome function

scientific article published on 23 January 2007

The human mitochondrial replication fork in health and disease

scientific article published on 22 April 2010

The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria

scientific article published on 18 April 2013

The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis

scientific article

The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cells

scientific article

Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA.

scientific article published on 14 December 2017

Unraveling the structure of DNA during overstretching by using multicolor, single-molecule fluorescence imaging

scientific article

List of works by Maria Falkenberg

A family of putative transcription termination factors shared amongst metazoans and plants

A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop

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

Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing

Adenosine kinase deficiency disrupts the methionine cycle and causes hypermethioninemia, encephalopathy, and abnormal liver function

Characterization of the mouse genes for mitochondrial transcription factors B1 and B2.

Cloning of the sea urchin mitochondrial RNA polymerase and reconstitution of the transcription termination system

Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies

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

DNA replication and transcription in mammalian mitochondria

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

Human mitochondrial transcription revisited: only TFAM and TFB2M are required for transcription of the mitochondrial genes in vitro

In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication

MGME1 processes flaps into ligatable nicks in concert with DNA polymerase γ during mtDNA replication

MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation

MTERF3 is a negative regulator of mammalian mtDNA transcription

Maintenance and Expression of Mammalian Mitochondrial DNA.

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription

Mammalian mitochondrial DNA replication and mechanisms of deletion formation

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery

Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria.

Mitochondrial DNA replication in mammalian cells: overview of the pathway

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

Mitochondrial transcription termination factor 1 directs polar replication fork pausing

POLRMT mutations impair mitochondrial transcription causing neurological disease

Primer removal during mammalian mitochondrial DNA replication

RNase H1 directs origin-specific initiation of DNA replication in human mitochondria

Reconstitution of a minimal mtDNA replisome in vitro

Ribonucleotides embedded in template DNA impair mitochondrial RNA polymerase progression

Role of human DNA glycosylase Nei-like 2 (NEIL2) and single strand break repair protein polynucleotide kinase 3'-phosphatase in maintenance of mitochondrial genome

Selective mitochondrial DNA degradation following double-strand breaks

Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia

Small-molecule inhibitors of human mitochondrial DNA transcription

Structural basis for adPEO-causing mutations in the mitochondrial TWINKLE helicase

Structure-function defects of the TWINKLE linker region in progressive external ophthalmoplegia

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].

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

TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication

The DNA Ligands Influence the Interactions between the Herpes Simplex Virus 1 Origin Binding Protein and the Single Strand DNA-binding Protein, ICP-8 ⬇️

The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase activities

The UL8 Subunit of the Heterotrimeric Herpes Simplex Virus Type 1 Helicase-Primase Is Required for the Unwinding of Single Strand DNA-binding Protein (ICP8)-coated DNA Substrates ⬇️

The UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymes

The accessory subunit B of DNA polymerase gamma is required for mitochondrial replisome function

The human mitochondrial replication fork in health and disease

The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria

The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis

The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cells

Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA.

Unraveling the structure of DNA during overstretching by using multicolor, single-molecule fluorescence imaging