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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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REXO2 is an oligoribonuclease active in human mitochondria

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

2013

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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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Salvaging hope: Is increasing NAD+ a key to treating mitochondrial myopathy?

EMBO molecular medicine, 2014-06, Vol.6 (6), p.705-707

2014

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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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Pathogenic variants in HTRA2 cause an early-onset mitochondrial syndrome associated with 3-methylglutaconic aciduria

Journal of inherited metabolic disease, 2017-01, Vol.40 (1), p.121-130

2017

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Defective Mitochondrial mRNA Maturation Is Associated with Spastic Ataxia

American journal of human genetics, 2010-11, Vol.87 (5), p.655-660

2010

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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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Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency

Brain (London, England : 1878), 2011-01, Vol.134 (Pt 1), p.183-195

2011

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The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families

Brain (London, England : 1878), 2010-10, Vol.133 (10), p.2952-2963

2010

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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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SLIRP stabilizes LRPPRC via an RRM-PPR protein interface

Nucleic acids research, 2016-08, Vol.44 (14), p.6868

2016

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Structure based hypothesis of a mitochondrial ribosome rescue mechanism

Biology direct, 2012-05, Vol.7 (1), p.14-14

2012

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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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Clinical, biochemical, and genetic features associated with VARS2‐related mitochondrial disease

Human mutation, 2018-04, Vol.39 (4), p.563-578

2018

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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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The human mitochondrial ribosome recycling factor is essential for cell viability

Nucleic acids research, 2008-10, Vol.36 (18), p.5787

2008

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Human pentatricopeptide proteins: Only a few and what do they do?

RNA biology, 2013, Vol.10 (9), p.1433-1438

2013

Volltextzugriff (PDF)

How much does a disrupted mitochondrial network influence neuronal dysfunction?

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

2019

Volltextzugriff (PDF)

The role of the mitochondrial ribosome in human disease: searching for mutations in 12S mitochondrial rRNA with high disruptive potential

Human molecular genetics, 2014-02, Vol.23 (4), p.949-967

2014

Volltextzugriff (PDF)

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

Brain (London, England : 1878), 2009-11, Vol.132 (11), p.3165-3174

2009

Volltextzugriff (PDF)

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

Brain (London, England : 1878), 2002-07, Vol.125 (7), p.1522-1533

2002

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Redecorating the Mitochondrial Inner Membrane: A Treatment for mtDNA Disorders

Molecular therapy, 2020-08, Vol.28 (8), p.1749-1751

2020

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Clinical and molecular characterization of novel FARS2 variants causing neonatal mitochondrial disease

Molecular genetics and metabolism, 2023-11, Vol.140 (3), p.107657, Article 107657

2023

Volltextzugriff (PDF)

Using mitoribosomal profiling to investigate human mitochondrial translation

Wellcome open research, 2018-01, Vol.2, p.116-116

2018

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The process of mammalian mitochondrial protein synthesis

REXO2 is an oligoribonuclease active in human mitochondria

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

Salvaging hope: Is increasing NAD+ a key to treating mitochondrial myopathy?

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

Pathogenic variants in HTRA2 cause an early-onset mitochondrial syndrome associated with 3-methylglutaconic aciduria

Defective Mitochondrial mRNA Maturation Is Associated with Spastic Ataxia

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

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency

The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

SLIRP stabilizes LRPPRC via an RRM-PPR protein interface

Structure based hypothesis of a mitochondrial ribosome rescue mechanism

OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect

Clinical, biochemical, and genetic features associated with VARS2‐related mitochondrial disease

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

The human mitochondrial ribosome recycling factor is essential for cell viability

Human pentatricopeptide proteins: Only a few and what do they do?

How much does a disrupted mitochondrial network influence neuronal dysfunction?

The role of the mitochondrial ribosome in human disease: searching for mutations in 12S mitochondrial rRNA with high disruptive potential

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

Redecorating the Mitochondrial Inner Membrane: A Treatment for mtDNA Disorders

Clinical and molecular characterization of novel FARS2 variants causing neonatal mitochondrial disease

Using mitoribosomal profiling to investigate human mitochondrial translation

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