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Mitochondrial Diseases: Hope for the Future

Cell, 2020-04, Vol.181 (1), p.168-188

2020

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Mutations causing mitochondrial disease: What is new and what challenges remain?

Science (American Association for the Advancement of Science), 2015-09, Vol.349 (6255), p.1494-1499

2015

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The process of mammalian mitochondrial protein synthesis

Cell and tissue research, 2017-01, Vol.367 (1), p.5-20

2017

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High-resolution imaging reveals compartmentalization of mitochondrial protein synthesis in cultured human cells

Proceedings of the National Academy of Sciences - PNAS, 2021-02, Vol.118 (6)

2021

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Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit

Biochemical journal, 2010-09, Vol.430 (3), p.551-558

2010

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GRSF1 Regulates RNA Processing in Mitochondrial RNA Granules

Cell metabolism, 2013-03, Vol.17 (3), p.399-410

2013

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Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

Human molecular genetics, 2018-05, Vol.27 (10), p.1743-1753

2018

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Mitochondrial protein synthesis: Figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation

FEBS letters, 2014-08, Vol.588 (15), p.2496-2503

2014

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The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules

The Journal of biological chemistry, 2017-03, Vol.292 (11), p.4519-4532

2017

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REXO2 is an oligoribonuclease active in human mitochondria

PloS one, 2013-05, Vol.8 (5), p.e64670-e64670

2013

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Rescuing stalled mammalian mitoribosomes - what can we learn from bacteria?

Journal of cell science, 2020-01, Vol.133 (1)

2020

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Human mitochondrial nucleases

The FEBS journal, 2017-06, Vol.284 (12), p.1767-1777

2017

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OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect

EMBO molecular medicine, 2018-11, Vol.10 (11), p.n/a

2018

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The human RNA-binding protein RBFA promotes the maturation of the mitochondrial ribosome

Biochemical journal, 2017-06, Vol.474 (13), p.2145-2158

2017

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Hungry Codons Promote Frameshifting in Human Mitochondrial Ribosomes

Science (American Association for the Advancement of Science), 2010-01, Vol.327 (5963), p.301-301

2010

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Pronuclear transfer in human embryos to prevent transmission of mitochondrial DNA disease

Nature (London), 2010-05, Vol.465 (7294), p.82-85

2010

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Mammalian mitochondrial translation - revealing consequences of divergent evolution

Biochemical Society transactions, 2019-10, Vol.47 (5), p.1429-1436

2019

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

Biochimica et biophysica acta, 2014-01, Vol.1842 (1), p.56-64

2014

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DNA repair in organelles: Pathways, organization, regulation, relevance in disease and aging

Biochimica et biophysica acta, 2011, Vol.1813 (1), p.186-200

2011

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Cl-out is a novel cooperative optogenetic tool for extruding chloride from neurons

Nature communications, 2016-11, Vol.7 (1), p.13495-13495, Article 13495

2016

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How much does a disrupted mitochondrial network influence neuronal dysfunction?

EMBO molecular medicine, 2019-01, Vol.11 (1), p.n/a

2019

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Advances in methods for reducing mitochondrial DNA disease by replacing or manipulating the mitochondrial genome

Essays in biochemistry, 2018-07, Vol.62 (3), p.455

2018

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Termination of Protein Synthesis in Mammalian Mitochondria

The Journal of biological chemistry, 2011-10, Vol.286 (40), p.34479-34485

2011

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Mitochondrial transplantation-a possible therapeutic for mitochondrial dysfunction?: Mitochondrial transfer is a potential cure for many diseases but proof of efficacy and safety is still lacking

EMBO reports, 2020-09, Vol.21 (9), p.e50964-e50964

2020

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The role of TDP1 and APTX in mitochondrial DNA repair

Biochimie, 2014-05, Vol.100, p.121-124

2014

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Mitochondrial Diseases: Hope for the Future

Mutations causing mitochondrial disease: What is new and what challenges remain?

The process of mammalian mitochondrial protein synthesis

High-resolution imaging reveals compartmentalization of mitochondrial protein synthesis in cultured human cells

Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit

GRSF1 Regulates RNA Processing in Mitochondrial RNA Granules

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

Mitochondrial protein synthesis: Figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation

The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules

REXO2 is an oligoribonuclease active in human mitochondria

Rescuing stalled mammalian mitoribosomes - what can we learn from bacteria?

Human mitochondrial nucleases

OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect

The human RNA-binding protein RBFA promotes the maturation of the mitochondrial ribosome

Hungry Codons Promote Frameshifting in Human Mitochondrial Ribosomes

Pronuclear transfer in human embryos to prevent transmission of mitochondrial DNA disease

Mammalian mitochondrial translation - revealing consequences of divergent evolution

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

DNA repair in organelles: Pathways, organization, regulation, relevance in disease and aging

Cl-out is a novel cooperative optogenetic tool for extruding chloride from neurons

How much does a disrupted mitochondrial network influence neuronal dysfunction?

Advances in methods for reducing mitochondrial DNA disease by replacing or manipulating the mitochondrial genome

Termination of Protein Synthesis in Mammalian Mitochondria

Mitochondrial transplantation-a possible therapeutic for mitochondrial dysfunction?: Mitochondrial transfer is a potential cure for many diseases but proof of efficacy and safety is still lacking

The role of TDP1 and APTX in mitochondrial DNA repair

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