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Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)

Proceedings of the National Academy of Sciences - PNAS, 2010-10, Vol.107 (43), p.18410-18415

2010

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Sphingolipid lysosomal storage diseases: from bench to bedside

Lipids in health and disease, 2021-05, Vol.20 (1), p.44-44, Article 44

2021

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Mechanism of strand displacement DNA synthesis by the coordinated activities of human mitochondrial DNA polymerase and SSB

Nucleic acids research, 2023-02, Vol.51 (4), p.1750-1765

2023

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A second hybrid-binding domain modulates the activity of Drosophila ribonuclease H1

Journal of biochemistry (Tokyo), 2020-11, Vol.168 (5), p.515-533

2020

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DNA synthesis determines the binding mode of the human mitochondrial single-stranded DNA-binding protein

Nucleic acids research, 2017-07, Vol.45 (12), p.7237-7248

2017

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Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation

Scientific reports, 2017-08, Vol.7 (1), p.8315-14, Article 8315

2017

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Implications of Membrane Binding by the Fe-S Cluster-Containing N-Terminal Domain in the Drosophila Mitochondrial Replicative DNA Helicase

Frontiers in genetics, 2021-12, Vol.12, p.790521-790521

2021

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Physical and Functional Interaction of Mitochondrial Single-Stranded DNA-Binding Protein and the Catalytic Subunit of DNA Polymerase Gamma

Frontiers in genetics, 2021-09, Vol.12, p.721864-721864

2021

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Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein

PloS one, 2010-10, Vol.5 (10), p.e15379-e15379

2010

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Mitochondrial Protein Synthesis and mtDNA Levels Coordinated through an Aminoacyl-tRNA Synthetase Subunit

Cell reports (Cambridge), 2019-04, Vol.27 (1), p.40-47.e5

2019

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Mitochondrial chaperone TRAP1 activates the mitochondrial UPR and extends healthspan in Drosophila

Mechanisms of ageing and development, 2014-11, Vol.141-142, p.35-45

2014

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Lethal Interaction of Nuclear and Mitochondrial Genotypes in Drosophila melanogaster

G3 : genes - genomes - genetics, 2019-07, Vol.9 (7), p.2225-2234

2019

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Differential Phenotypes of Active Site and Human Autosomal Dominant Progressive External Ophthalmoplegia Mutations in Drosophila Mitochondrial DNA Helicase Expressed in Schneider Cells

The Journal of biological chemistry, 2007-03, Vol.282 (13), p.9436-9444

2007

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Iron-sulfur clusters in mitochondrial metabolism: Multifaceted roles of a simple cofactor

Biochemistry, 2016-10, Vol.81 (10), p.1066-1080

2016

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Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects

PloS one, 2012-08, Vol.7 (8), p.e43954-e43954

2012

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Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo

PloS one, 2009-11, Vol.4 (11), p.e7874-e7874

2009

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Drosophila Mitochondrial Transcription Factor B2 Regulates Mitochondrial DNA Copy Number and Transcription in Schneider Cells

The Journal of biological chemistry, 2004-06, Vol.279 (26), p.26900-26905

2004

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Coiled Coil Domain-containing Protein 56 (CCDC56) Is a Novel Mitochondrial Protein Essential for Cytochrome c Oxidase Function

The Journal of biological chemistry, 2012-07, Vol.287 (29), p.24174-24185

2012

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DNA polymerase gamma, the mitochondrial replicase

Annual review of biochemistry, 2004-01, Vol.73 (1), p.293-320

Kaguni, Laurie S

2004

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Drosophila Mitochondrial Transcription Factor B1 Modulates Mitochondrial Translation but Not Transcription or DNA Copy Number in Schneider Cells

The Journal of biological chemistry, 2005-04, Vol.280 (17), p.16815-16820

2005

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Matrix proteases in mitochondrial DNA function

Biochimica et biophysica acta, 2012-09, Vol.1819 (9-10), p.1080-1087

2012

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Physiological and Biochemical Defects in Carboxyl-terminal Mutants of Mitochondrial DNA Helicase

The Journal of biological chemistry, 2008-08, Vol.283 (35), p.23964-23971

2008

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Mapping 136 pathogenic mutations into functional modules in human DNA polymerase γ establishes predictive genotype–phenotype correlations for the complete spectrum of POLG syndromes

Biochimica et biophysica acta, 2014-07, Vol.1837 (7), p.1113-1121

2014

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Replicative DNA polymerases promote active displacement of SSB proteins during lagging strand synthesis

Nucleic acids research, 2019-06, Vol.47 (11), p.5723-5734

2019

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Reduced Stimulation of Recombinant DNA Polymerase γ and Mitochondrial DNA (mtDNA) Helicase by Variants of Mitochondrial Single-stranded DNA-binding Protein (mtSSB) Correlates with Defects in mtDNA Replication in Animal Cells

The Journal of biological chemistry, 2011-11, Vol.286 (47), p.40649-40658

2011

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Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)

Sphingolipid lysosomal storage diseases: from bench to bedside

Mechanism of strand displacement DNA synthesis by the coordinated activities of human mitochondrial DNA polymerase and SSB

A second hybrid-binding domain modulates the activity of Drosophila ribonuclease H1

DNA synthesis determines the binding mode of the human mitochondrial single-stranded DNA-binding protein

Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation

Implications of Membrane Binding by the Fe-S Cluster-Containing N-Terminal Domain in the Drosophila Mitochondrial Replicative DNA Helicase

Physical and Functional Interaction of Mitochondrial Single-Stranded DNA-Binding Protein and the Catalytic Subunit of DNA Polymerase Gamma

Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein

Mitochondrial Protein Synthesis and mtDNA Levels Coordinated through an Aminoacyl-tRNA Synthetase Subunit

Mitochondrial chaperone TRAP1 activates the mitochondrial UPR and extends healthspan in Drosophila

Lethal Interaction of Nuclear and Mitochondrial Genotypes in Drosophila melanogaster

Differential Phenotypes of Active Site and Human Autosomal Dominant Progressive External Ophthalmoplegia Mutations in Drosophila Mitochondrial DNA Helicase Expressed in Schneider Cells

Iron-sulfur clusters in mitochondrial metabolism: Multifaceted roles of a simple cofactor

Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects

Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo

Drosophila Mitochondrial Transcription Factor B2 Regulates Mitochondrial DNA Copy Number and Transcription in Schneider Cells

Coiled Coil Domain-containing Protein 56 (CCDC56) Is a Novel Mitochondrial Protein Essential for Cytochrome c Oxidase Function

DNA polymerase gamma, the mitochondrial replicase

Drosophila Mitochondrial Transcription Factor B1 Modulates Mitochondrial Translation but Not Transcription or DNA Copy Number in Schneider Cells

Matrix proteases in mitochondrial DNA function

Physiological and Biochemical Defects in Carboxyl-terminal Mutants of Mitochondrial DNA Helicase

Mapping 136 pathogenic mutations into functional modules in human DNA polymerase γ establishes predictive genotype–phenotype correlations for the complete spectrum of POLG syndromes

Replicative DNA polymerases promote active displacement of SSB proteins during lagging strand synthesis

Reduced Stimulation of Recombinant DNA Polymerase γ and Mitochondrial DNA (mtDNA) Helicase by Variants of Mitochondrial Single-stranded DNA-binding Protein (mtSSB) Correlates with Defects in mtDNA Replication in Animal Cells

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