List of works - Nils-Göran Larsson

A common variant in TFB1M is associated with reduced insulin secretion and increased future risk of type 2 diabetes

scientific article published on January 2011

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

scientific article published on October 2005

A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts

scholarly article

Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy

scientific article

Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing

scientific article published on 14 December 2020

Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance

scientific article published on 15 November 2012

Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration

scientific article

Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons

scientific article published on 16 November 2011

An Adaptable High-Throughput Technology Enabling the Identification of Specific Transcription Modulators

scientific article published on 31 January 2017

Animal models for respiratory chain disease ⬇️

scientific article published on December 1, 2001

Atypical presentation of multisystem disorders in two girls with mitochondrial DNA deletions

scientific article published in January 1995

Author Correction: MitoTALEN reduces mutant mtDNA load and restores tRNA levels in a mouse model of heteroplasmic mtDNA mutation

scientific article published on 01 December 2018

Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy

scientific article published on 01 April 1994

Cellular pyrimidine imbalance triggers mitochondrial DNA-dependent innate immunity

scientific article published on 26 April 2021

Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila

scientific article published on 12 July 2017

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

scientific article

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

scientific article published on 10 November 2015

Complete Deletion of a POLG1 Allele in a Patient with Alpers Syndrome

scientific article published on 20 October 2011

Complex genetic counselling and prenatal analysis in a woman with external ophthalmoplegia and deleted mtDNA

scientific article published on 01 May 2000

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

De novo mutation in the mitochondrial ATP synthase subunit 6 gene (T8993G) with rapid segregation resulting in Leigh syndrome in the offspring

scientific article published on 01 September 1995

Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression

scientific article published in January 1999

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

scientific article published on 03 October 2019

Down-regulation of mitochondrial transcription factor A during spermatogenesis in humans

scientific article

Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis

scientific article published in October 2001

FBXL4 deficiency increases mitochondrial removal by autophagy

scientific article published on 11 June 2020

Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne.

scientific article published in March 1996

Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy

scientific article

Germline mitochondrial DNA mutations aggravate ageing and can impair brain development

scientific article published on 21 August 2013

High brain lactate is a hallmark of aging and caused by a shift in the lactate dehydrogenase A/B ratio

scientific article

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo

scholarly article

Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes.

scientific article published in November 2000

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo

scientific article

Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease

scientific article

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

scientific article published on 23 October 2012

Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load

scientific article

Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy

scientific article

Increased mitochondrial mass in mitochondrial myopathy mice

scientific article

Inheritance and expression of mitochondrial DNA point mutations

scientific article published on 01 May 1995

Keeping mtDNA in shape between generations

scientific article

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

scientific article published on 13 July 2010

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

scientific article

Lack of transmission of deleted mtDNA from a woman with Kearns-Sayre syndrome to her child ⬇️

scientific article published on February 1, 1992

Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.

scientific article

Leber's hereditary optic neuropathy and complex I deficiency in muscle

scientific article published in November 1991

Loss of LRPPRC causes ATP synthase deficiency

scientific article published on 6 January 2014

Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion

scientific article published on May 1994

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

scientific journal article

MTERF1 gives mtDNA an unusual twist

scientific article published on 01 July 2010

MTERF2 is a nucleoid component in mammalian mitochondria

scientific article

MTERF3 is a negative regulator of mammalian mtDNA transcription

scientific article

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals

scientific article

MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome

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

Making proteins in the powerhouse

scientific article published on 31 July 2014

Mammalian Mitochondria and Aging: An Update

scientific article published on 27 October 2016

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

scientific article

Manipulation of mitochondrial DNA gene expression in the mouse

scientific article

Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome

scientific article

Mic10 Oligomerization Pinches off Mitochondrial Cristae

scientific article published on May 2015

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

scientific article published on 23 March 2018

MitoTALEN reduces mutant mtDNA load and restores tRNAAla levels in a mouse model of heteroplasmic mtDNA mutation

scientific article published on 24 September 2018

Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria

scientific article (publication date: December 1992)

Mitochondrial DNA copy number in human disease: the more the better?

scientific article published on 12 December 2020

Mitochondrial DNA deletions and cytochrome c oxidase deficiency in muscle fibres

scientific article published on 01 July 1992

Mitochondrial DNA deletions in inclusion body myositis

scientific article published in April 1993

Mitochondrial DNA deletions in muscle fibers in inclusion body myositis

scientific article published on 01 July 1995

Mitochondrial DNA mutations in disease and aging

scientific article

Mitochondrial dysfunction in mammalian ageing.

scientific article published on January 2007

Mitochondrial encephalomyopathies in childhood. I. Biochemical and morphologic investigations

scientific article published on 01 August 1991

Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes

scientific article published on August 1991

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

scientific article published on 06 June 2019

Mitochondrial medicine--recent advances

scientific article

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice

scientific article (publication date: March 1998)

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

Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons

scientific article published on 21 August 2012

Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels.

scientific article

MtDNA mutations are a common cause of severe disease phenotypes in children with Leigh syndrome

scientific article

Multiple short direct repeats associated with single mtDNA deletions

scientific article published on 01 August 1992

Multiple symmetric lipomas with high levels of mtDNA with the tRNA(Lys) A-->G(8344) mutation as the only manifestation of disease in a carrier of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome.

scientific article

NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly

scientific article

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

scientific article published on 28 August 2020

Neuropathology in Kearns-Sayre syndrome

scientific article published on January 1990

Overexpression of the mitochondrial methyltransferase TFB1M in the mouse does not impact mitoribosomal methylation status or hearing

scientific article

POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA.

scientific article published on 05 August 2016

Pathogenetic aspects of the A8344G mutation of mitochondrial DNA associated with MERRF syndrome and multiple symmetric lipomas

scientific article published on 01 January 1995

Progressive increase of the mutated mitochondrial DNA fraction in Kearns-Sayre syndrome

scientific article published on 01 August 1990

Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons

scholarly article

Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos

scientific article published on 10 August 2020

Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology

scientific article published on 26 September 2006

Random point mutations with major effects on protein-coding genes are the driving force behind premature aging in mtDNA mutator mice

scientific article

Reactive oxygen species and fatigue-induced prolonged low-frequency force depression in skeletal muscle fibres of rats, mice and SOD2 overexpressing mice

scientific article published on 15 November 2007

Regulation of mitochondrial DNA copy number during spermatogenesis

scientific article

Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance

scientific article

Role of the mitochondrial protein LRPPRC2 in Drosophila melanogaster

S10.25 Effect of targeted quinones on ROS production and lipid peroxidation in mitochondria: Mitochondrial DNA polymerase mutant mice exibit high sensitivity

S14.4 Random mtDNA mutations cause respiratory dysfunction through failure in complex assembly

S14.6 Impaired thermogenesis in PolgA mtDNA polymerase mutant mice

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation

scientific article published on 6 August 2015

SOD2 overexpression: enhanced mitochondrial tolerance but absence of effect on UCP activity

scientific article published on 10 November 2005

Secondary metabolic effects in complex I deficiency

scientific article published in October 2005

Segregation and manifestations of the mtDNA tRNA(Lys) A-->G(8344) mutation of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome

scientific article

Sensory ataxic neuropathy in golden retriever dogs is caused by a deletion in the mitochondrial tRNATyr gene

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

SnapShot: Mitochondrial Nucleoid

scientific article published in January 2018

Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production

scientific article

Strong purifying selection in transmission of mammalian mitochondrial DNA

scientific article

Structure and chromosomal localization of the mouse mitochondrial transcription factor A gene (Tfam).

scientific article published in February 1997

Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis

scientific article

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

scientific article

TEFM regulates both transcription elongation and RNA processing in mitochondria.

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 Enigma of the Respiratory Chain Supercomplex

scientific article published on April 2017

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

scientific journal article

The amino terminal extension of mammalian mitochondrial RNA polymerase ensures promoter specific transcription initiation

scientific article published on 20 January 2014

The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster

scientific article

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

scientific article published on 18 April 2013

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

scientific article

The respiratory chain supercomplex organization is independent of COX7a2l isoforms

scientific article

The role of mitochondria in aging

scientific article published on March 2013

The role of mitochondrial DNA mutations and free radicals in disease and ageing ⬇️

scientific article published on March 7, 2013

The transcription machinery in mammalian mitochondria

scientific article

Tissue distribution and disease manifestations of the tRNA(Lys) A-->G(8344) mitochondrial DNA mutation in a case of myoclonus epilepsy and ragged red fibres

scientific article published on 01 January 1995

Tissue-specific knockout model for study of mitochondrial DNA mutation disorders

scientific article

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

scientific article published on 14 December 2017

Tracing the trail of protons through complex I of the mitochondrial respiratory chain

scientific article

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

scientific article published on 14 November 2017

Two novel mutations in thymidine kinase-2 cause early onset fatal encephalomyopathy and severe mtDNA depletion

scientific article published on 18 January 2010

Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins

scientific article

Understanding aging: revealing order out of chaos

scientific article published in July 2004

Unraveling the biological roles of reactive oxygen species

scientific article published on April 2011

Use of primary cultures and continuous cell lines to study effects on astrocytic regulatory functions ⬇️

scientific article published on April 1, 1995

Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission

scientific article

[DNA diagnosis of mitochondrial diseases is now possible]

scientific article published on 01 November 1989

List of works by Nils-Göran Larsson

A common variant in TFB1M is associated with reduced insulin secretion and increased future risk of type 2 diabetes

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

A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts

Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy

Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing

Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance

Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration

Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons

An Adaptable High-Throughput Technology Enabling the Identification of Specific Transcription Modulators

Animal models for respiratory chain disease ⬇️

Atypical presentation of multisystem disorders in two girls with mitochondrial DNA deletions

Author Correction: MitoTALEN reduces mutant mtDNA load and restores tRNA levels in a mouse model of heteroplasmic mtDNA mutation

Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy

Cellular pyrimidine imbalance triggers mitochondrial DNA-dependent innate immunity

Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila

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

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

Complete Deletion of a POLG1 Allele in a Patient with Alpers Syndrome

Complex genetic counselling and prenatal analysis in a woman with external ophthalmoplegia and deleted mtDNA

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

DNA replication and transcription in mammalian mitochondria

De novo mutation in the mitochondrial ATP synthase subunit 6 gene (T8993G) with rapid segregation resulting in Leigh syndrome in the offspring

Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

Down-regulation of mitochondrial transcription factor A during spermatogenesis in humans

Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis

FBXL4 deficiency increases mitochondrial removal by autophagy

Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne.

Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy

Germline mitochondrial DNA mutations aggravate ageing and can impair brain development

High brain lactate is a hallmark of aging and caused by a shift in the lactate dehydrogenase A/B ratio

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo

Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes.

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo

Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease

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

Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load

Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy

Increased mitochondrial mass in mitochondrial myopathy mice

Inheritance and expression of mitochondrial DNA point mutations

Keeping mtDNA in shape between generations

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

Lack of transmission of deleted mtDNA from a woman with Kearns-Sayre syndrome to her child ⬇️

Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.

Leber's hereditary optic neuropathy and complex I deficiency in muscle

Loss of LRPPRC causes ATP synthase deficiency

Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion

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

MTERF1 gives mtDNA an unusual twist

MTERF2 is a nucleoid component in mammalian mitochondria

MTERF3 is a negative regulator of mammalian mtDNA transcription

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals

MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome

Maintenance and Expression of Mammalian Mitochondrial DNA.

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

Making proteins in the powerhouse

Mammalian Mitochondria and Aging: An Update

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

Manipulation of mitochondrial DNA gene expression in the mouse

Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome

Mic10 Oligomerization Pinches off Mitochondrial Cristae

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

MitoTALEN reduces mutant mtDNA load and restores tRNAAla levels in a mouse model of heteroplasmic mtDNA mutation

Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria

Mitochondrial DNA copy number in human disease: the more the better?

Mitochondrial DNA deletions and cytochrome c oxidase deficiency in muscle fibres

Mitochondrial DNA deletions in inclusion body myositis

Mitochondrial DNA deletions in muscle fibers in inclusion body myositis

Mitochondrial DNA mutations in disease and aging

Mitochondrial dysfunction in mammalian ageing.

Mitochondrial encephalomyopathies in childhood. I. Biochemical and morphologic investigations

Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

Mitochondrial medicine--recent advances

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons