(Q37785521)

23 August 2017

title

Emerging therapeutic approaches to mitochondrial diseases (English)

1 reference

23 August 2017

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Tina Wenz

1

1 reference

23 August 2017

Sion L. Williams

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20818736

7 July 2024

Sandra R. Bacman

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20818736

7 July 2024

Carlos T. Moraes

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20818736

7 July 2024

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=20818736

7 July 2024

publication date

1 January 2010

1 reference

full work available at URL

23 August 2017

https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1002%2Fddrr.109

1 reference

https://onlinelibrary.wiley.com/doi/pdf/10.1002/ddrr.109

7 July 2024

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Developmental Disabilities Research Reviews

7 July 2024

published in

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23 August 2017

volume

16

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23 August 2017

issue

2

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23 August 2017

page(s)

219-229

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Evaluation of bupivacaine-induced muscle regeneration in the treatment of ptosis in patients with chronic progressive external ophthalmoplegia and Kearns-Sayre syndrome

23 August 2017

cites work

1 reference

The inheritance of pathogenic mitochondrial DNA mutations

21 January 2018

1 reference

A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases

21 January 2018

1 reference

Biochemical, physiological and medical aspects of ubiquinone function

21 January 2018

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Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation

21 January 2018

1 reference

Multiple triphenylphosphonium cations shuttle a hydrophilic peptide into mitochondria.

21 January 2018

1 reference

7 January 2021

Modulation of mitochondrial protein phosphorylation by soluble adenylyl cyclase ameliorates cytochrome oxidase defects

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7 January 2021

Cyclic AMP produced inside mitochondria regulates oxidative phosphorylation

1 reference

7 January 2021

Targeting an antioxidant to mitochondria decreases cardiac ischemia-reperfusion injury

1 reference

7 January 2021

Ketogenic diet slows down mitochondrial myopathy progression in mice

1 reference

7 January 2021

Selective elimination of mutant mitochondrial genomes as therapeutic strategy for the treatment of NARP and MILS syndromes

1 reference

7 January 2021

Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients

1 reference

7 January 2021

Organ-specific shifts in mtDNA heteroplasmy following systemic delivery of a mitochondria-targeted restriction endonuclease

1 reference

7 January 2021

Modulating mtDNA heteroplasmy by mitochondria-targeted restriction endonucleases in a 'differential multiple cleavage-site' model

1 reference

7 January 2021

Mitochondria, oxidants, and aging

1 reference

7 January 2021

Neuroprotective effect of long-term NDI1 gene expression in a chronic mouse model of Parkinson disorder

1 reference

7 January 2021

Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components

1 reference

7 January 2021

Rapid directional shift of mitochondrial DNA heteroplasmy in animal tissues by a mitochondrially targeted restriction endonuclease

1 reference

7 January 2021

TAT-mediated protein transduction into mammalian cells

1 reference

7 January 2021

PGC-1alpha-mediated regulation of gene expression and metabolism: implications for nutrition and exercise prescriptions

1 reference

7 January 2021

The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes

1 reference

7 January 2021

A calcium signaling defect in the pathogenesis of a mitochondrial DNA inherited oxidative phosphorylation deficiency

1 reference

7 January 2021

Transmission of mitochondrial DNA disorders: possibilities for the future.

1 reference

7 January 2021

Targeting lipoic acid to mitochondria: synthesis and characterization of a triphenylphosphonium-conjugated alpha-lipoyl derivative

1 reference

7 January 2021

Progress and prospects: zinc-finger nucleases as gene therapy agents

1 reference

7 January 2021

Reversal of a mitochondrial DNA defect in human skeletal muscle

1 reference

7 January 2021

Mitochondrial targeting of quinones: therapeutic implications.

1 reference

7 January 2021

Birth of infant after transfer of anucleate donor oocyte cytoplasm into recipient eggs

1 reference

7 January 2021

The alternative oxidase, a tool for compensating cytochrome c oxidase deficiency in human cells.

1 reference

7 January 2021

Expression of the alternative oxidase complements cytochrome c oxidase deficiency in human cells

1 reference

7 January 2021

Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease.

1 reference

7 January 2021

Targeting proteins to mitochondria using TAT.

1 reference

7 January 2021

A novel TAT-mitochondrial signal sequence fusion protein is processed, stays in mitochondria, and crosses the placenta

1 reference

7 January 2021

Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency

1 reference

7 January 2021

Mitochondrial respiratory-chain diseases

1 reference

7 January 2021

Mitochondria biogenesis induced by resveratrol against brain ischemic stroke

1 reference

7 January 2021

Optimized allotopic expression of the human mitochondrial ND4 prevents blindness in a rat model of mitochondrial dysfunction

1 reference

7 January 2021

MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination

1 reference

7 January 2021

The Interaction of Q Analogs, Particularly Hydroxydecyl Benzoquinone (Idebenone), with the Respiratory Complexes of Heart Mitochondria

1 reference

7 January 2021

Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation

1 reference

7 January 2021

Radical medicine: treating ageing to cure disease

1 reference

7 January 2021

Adeno-associated virus-mediated gene transfer of the heart/muscle adenine nucleotide translocator (ANT) in mouse

1 reference

7 January 2021

Transmission of mitochondrial DNA disorders: possibilities for the elimination of mutated mitochondria

1 reference

7 January 2021

A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator

1 reference

7 January 2021

Allotopic expression of mitochondrial ATP synthase genes in nucleus of Saccharomyces cerevisiae

1 reference

7 January 2021

Efficiency and safety of AAV-mediated gene delivery of the human ND4 complex I subunit in the mouse visual system

1 reference

7 January 2021

The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease

1 reference

7 January 2021

Allotopic expression of a mitochondrial alternative oxidase confers cyanide resistance to human cell respiration

1 reference

7 January 2021

Drilling for energy in mitochondrial disease

1 reference

7 January 2021

Myogenic satellite cells: physiology to molecular biology

1 reference

7 January 2021

Allogeneic stem cell transplantation corrects biochemical derangements in MNGIE

1 reference

7 January 2021

Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression

1 reference

7 January 2021

Specific targeting of a DNA-alkylating reagent to mitochondria. Synthesis and characterization of [4-((11aS)-7-methoxy-1,2,3,11a-tetrahydro-5H-pyrrolo[2,1-c][1,4]benzodiazepin-5-on-8-oxy)butyl]-triphenylphosphonium iodide

1 reference

7 January 2021

Interactions of mitochondria-targeted and untargeted ubiquinones with the mitochondrial respiratory chain and reactive oxygen species. Implications for the use of exogenous ubiquinones as therapies and experimental tools

1 reference

7 January 2021

Tissue-specific selection for different mtDNA genotypes in heteroplasmic mice

1 reference

7 January 2021

Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects

1 reference

7 January 2021

Mitochondrial gene therapy augments mitochondrial physiology in a Parkinson's disease cell model

1 reference

7 January 2021

The discovery of zinc fingers and their applications in gene regulation and genome manipulation.

1 reference

7 January 2021

Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.

1 reference

7 January 2021

Scientists probe method to prevent inherited mitochondrial gene diseases

1 reference

7 January 2021

Progress and prospects: gene therapy for mitochondrial DNA disease

1 reference

7 January 2021

Infusion of platelets transiently reduces nucleoside overload in MNGIE

1 reference

7 January 2021

Human mitochondrial leucyl-tRNA synthetase corrects mitochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetes

1 reference

7 January 2021

Pathogenic mechanism of a human mitochondrial tRNAPhe mutation associated with myoclonic epilepsy with ragged red fibers syndrome.

1 reference

7 January 2021

Ketones inhibit mitochondrial production of reactive oxygen species production following glutamate excitotoxicity by increasing NADH oxidation

1 reference

7 January 2021

Functional delivery of a cytosolic tRNA into mutant mitochondria of human cells

1 reference

7 January 2021

Mitochondrial dysfunction, oxidative stress and neurodegeneration.

1 reference

7 January 2021

Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus

1 reference

7 January 2021

Protection by the NDI1 gene against neurodegeneration in a rotenone rat model of Parkinson's disease

1 reference

7 January 2021

Idebenone: an emerging therapy for Friedreich ataxia

1 reference

7 January 2021

Construction and testing of engineered zinc-finger proteins for sequence-specific modification of mtDNA

1 reference

7 January 2021

Sequence-specific modification of mitochondrial DNA using a chimeric zinc finger methylase

1 reference

7 January 2021

Development of a single-chain, quasi-dimeric zinc-finger nuclease for the selective degradation of mutated human mitochondrial DNA.

1 reference

7 January 2021

Muscle satellite cells

1 reference

7 January 2021

Sandwiched zinc-finger nucleases harboring a single-chain FokI dimer as a DNA-cleavage domain

1 reference

7 January 2021

Targeted mRNA degradation by complex-mediated delivery of antisense RNAs to intracellular human mitochondria

1 reference

7 January 2021

Resistance training in patients with single, large-scale deletions of mitochondrial DNA.

1 reference

7 January 2021

Drug delivery to mitochondria: the key to mitochondrial medicine

1 reference

7 January 2021

Oral administration of idebenone induces nerve growth factor in the brain and improves learning and memory in basal forebrain-lesioned rats

1 reference

7 January 2021

Neuroprotective effects of the ketogenic diet

1 reference

7 January 2021

Role of calcium and AMP kinase in the regulation of mitochondrial biogenesis and GLUT4 levels in muscle

1 reference

7 January 2021

The pathogenic A3243G mutation in human mitochondrial tRNALeu(UUR) decreases the efficiency of aminoacylation

1 reference

7 January 2021

Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene

1 reference

7 January 2021

Restoration of electron transport without proton pumping in mammalian mitochondria.

1 reference

7 January 2021

Establishment of HIV-1 resistance in CD4+ T cells by genome editing using zinc-finger nucleases

1 reference

7 January 2021

TAT-mediated delivery of LAD restores pyruvate dehydrogenase complex activity in the mitochondria of patients with LAD deficiency

1 reference

7 January 2021

Mitochondrial dysfunction and reactive oxygen species in excitotoxicity and apoptosis: implications for the pathogenesis of neurodegenerative diseases

1 reference

7 January 2021

Consequences of long-term oral administration of the mitochondria-targeted antioxidant MitoQ to wild-type mice.

1 reference

7 January 2021

Folate, mitochondria, ROS, and the aging brain

1 reference

7 January 2021

Respiratory chain alternative enzymes as tools to better understand and counteract respiratory chain deficiencies in human cells and animals

1 reference

7 January 2021

Ketogenic treatment reduces deleted mitochondrial DNAs in cultured human cells

1 reference

7 January 2021

The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2.

1 reference

7 January 2021

Transcriptional paradigms in mammalian mitochondrial biogenesis and function

1 reference

7 January 2021

The epidemiology of mitochondrial disorders--past, present and future

1 reference

7 January 2021

Clinical experience with high-dose idebenone in Friedreich ataxia

1 reference

7 January 2021

The single subunit NADH dehydrogenase reduces generation of reactive oxygen species from complex I.

1 reference

7 January 2021

A single-subunit NADH-quinone oxidoreductase renders resistance to mammalian nerve cells against complex I inhibition

1 reference

7 January 2021

Human NARP mitochondrial mutation metabolism corrected with alpha-ketoglutarate/aspartate: a potential new therapy

1 reference

7 January 2021

The mitochondrial cyanide-resistant oxidase: structural conservation amid regulatory diversity

1 reference

7 January 2021

Delivery of bioactive molecules to mitochondria in vivo.

1 reference

7 January 2021

PGC-1alpha/beta induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders.

1 reference

7 January 2021

Manipulating mitochondrial DNA heteroplasmy by a mitochondrially targeted restriction endonuclease

1 reference

7 January 2021

The role of the 3' untranslated region in mRNA sorting to the vicinity of mitochondria is conserved from yeast to human cells

1 reference

7 January 2021

Mitochondrial gene replacement in primate offspring and embryonic stem cells

1 reference

7 January 2021

Implications of exercise training in mtDNA defects--use it or lose it?

1 reference

7 January 2021

Exercise and training in mitochondrial myopathies

1 reference

7 January 2021

Import of nuclear DNA-encoded RNAs into mitochondria and mitochondrial translation.

1 reference

7 January 2021

Selective inhibition of mutant human mitochondrial DNA replication in vitro by peptide nucleic acids

1 reference

7 January 2021

Mitochondrial disorders: genetics, counseling, prenatal diagnosis and reproductive options.

1 reference

7 January 2021

TAT-mediated intracellular delivery of purine nucleoside phosphorylase corrects its deficiency in mice

1 reference

7 January 2021

Highly efficient endogenous human gene correction using designed zinc-finger nucleases

1 reference

7 January 2021

Inhibition of mitochondrial Na+-Ca2+ exchange restores agonist-induced ATP production and Ca2+ handling in human complex I deficiency.

1 reference

7 January 2021

Protein transduction: delivery of Tat-GTPase fusion proteins into mammalian cells

1 reference

7 January 2021

1 reference

7 January 2021

Protein transduction: cell penetrating peptides and their therapeutic applications

1 reference

7 January 2021

Mitochondrial neuro-gastrointestinal encephalopathy syndrome

1 reference

7 January 2021

Treatment of lactic acidosis.

1 reference

7 January 2021

PGC-1alpha activation as a therapeutic approach in mitochondrial disease

1 reference

7 January 2021

Endurance exercise is protective for mice with mitochondrial myopathy

1 reference

7 January 2021

RETRACTED: Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype

1 reference

7 January 2021

Regulation of mitochondrial biogenesis in skeletal muscle by CaMK

1 reference

7 January 2021

Transducer of regulated CREB-binding proteins (TORCs) induce PGC-1alpha transcription and mitochondrial biogenesis in muscle cells

1 reference

7 January 2021

Mitochondrial drug delivery and mitochondrial disease therapy--an approach to liposome-based delivery targeted to mitochondria

1 reference

7 January 2021

Limitations of Allotopic Expression of Mitochondrial Genes in Mammalian Cells

82

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Gene therapy for mitochondrial disease by delivering restriction endonuclease SmaI into mitochondria

7 July 2024

110

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7 July 2024

Identifiers

DOI

10.1002/DDRR.109

1 reference

23 August 2017

1 reference