Current strategies towards therapeutic manipulation of mtDNA heteroplasmy. (Q51344073)

PubMed publication ID

27814659

3 April 2018

http://europepmc.org/abstract/MED/27814659

review article

1 reference

title

Current strategies towards therapeutic manipulation of mtDNA heteroplasmy (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

1 reference

Claudia V Pereira

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

Carlos T Moraes

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

publication date

1 January 2017

1 reference

PubMed publication ID

27814659

3 April 2018

http://europepmc.org/abstract/MED/27814659

published in

Frontiers in Bioscience

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

volume

22

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

page(s)

991-1010

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

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

14 May 2020

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Correction of multi-gene deficiency in vivo using a single 'self-cleaving' 2A peptide-based retroviral vector

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A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies

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Breaking the DNA-binding code of Ralstonia solanacearum TAL effectors provides new possibilities to generate plant resistance genes against bacterial wilt disease

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A guide to genome engineering with programmable nucleases

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Histochemical and enzymatic comparison of the gastrocnemius muscle of young and elderly men and women

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Clinical spectrum of Leber's hereditary optic neuropathy.

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mtDNA depletion myopathy: elucidation of the tissue specificity in the mitochondrial thymidine kinase (TK2) deficiency

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Designer zinc-finger proteins and their applications

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A 2.2 A resolution crystal structure of a designed zinc finger protein bound to DNA

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Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome

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Mitochondrial DNA replication: what we know

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

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Nuclear genetic control of mitochondrial DNA segregation

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Introduction of disease-related mitochondrial DNA deletions into HeLa cells lacking mitochondrial DNA results in mitochondrial dysfunction

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Complete restoration of a wild-type mtDNA genotype in regenerating muscle fibres in a patient with a tRNA point mutation and mitochondrial encephalomyopathy

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Universal heteroplasmy of human mitochondrial DNA.

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Selective elimination of mitochondrial mutations in the germline by genome editing

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Ketogenic treatment reduces deleted mitochondrial DNAs in cultured human cells

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The dynamics of mitochondrial DNA heteroplasmy: implications for human health and disease

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MitoTALEN: A General Approach to Reduce Mutant mtDNA Loads and Restore Oxidative Phosphorylation Function in Mitochondrial Diseases

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Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNA(Lys) mutation

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Sequence homology at the breakpoint and clinical phenotype of mitochondrial DNA deletion syndromes

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12 December 2020

Promoter elements of rice susceptibility genes are bound and activated by specific TAL effectors from the bacterial blight pathogen, Xanthomonas oryzae pv. oryzae

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The mitochondrial DNA transfer RNA(Lys)A-->G(8344) mutation and the syndrome of myoclonic epilepsy with ragged red fibres (MERRF). Relationship of clinical phenotype to proportion of mutant mitochondrial DNA.

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The mitochondrial genome in human adaptive radiation and disease: on the road to therapeutics and performance enhancement

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

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12 December 2020

Estimation of Membrane Potential and pH Difference across the Cristae Membrane of Rat Liver Mitochondria

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12 December 2020

An improved zinc-finger nuclease architecture for highly specific genome editing

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12 December 2020

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

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12 December 2020

Efficient design and assembly of custom TALEN and other TAL effector-based constructs for DNA targeting

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12 December 2020

Sequence and organization of the human mitochondrial genome

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12 December 2020

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

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12 December 2020

A mouse model of mitochondrial disease reveals germline selection against severe mtDNA mutations

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12 December 2020

Pearson's marrow-pancreas syndrome. A multisystem mitochondrial disorder in infancy

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12 December 2020

Deletions of mitochondrial DNA in Kearns-Sayre syndrome

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12 December 2020

Nuclear and mitochondrial DNA repair: similar pathways?

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12 December 2020

Use of transmitochondrial cybrids to assign a complex I defect to the mitochondrial DNA-encoded NADH dehydrogenase subunit 6 gene mutation at nucleotide pair 14459 that causes Leber hereditary optic neuropathy and dystonia

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12 December 2020

Human mitochondrial DNA: roles of inherited and somatic mutations

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12 December 2020

A reduction of mitochondrial DNA molecules during embryogenesis explains the rapid segregation of genotypes

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12 December 2020

Thymidine phosphorylase mutations cause instability of mitochondrial DNA.

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12 December 2020

Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia

1 reference

12 December 2020

Extremely high levels of mutant mtDNAs co-localize with cytochrome c oxidase-negative ragged-red fibers in patients harboring a point mutation at nt 3243

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12 December 2020

Tissue-specific selection for different mtDNA genotypes in heteroplasmic mice

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12 December 2020

Leber hereditary optic neuropathy: clinical and molecular genetic findings

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12 December 2020

Enhancing zinc-finger-nuclease activity with improved obligate heterodimeric architectures

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12 December 2020

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

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12 December 2020

Ubiquitin-dependent proteolysis in mammalian spermatogenesis, fertilization, and sperm quality control: killing three birds with one stone

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12 December 2020

Recent evidence for evolution of the genetic code

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12 December 2020

Epigenetics, epidemiology and mitochondrial DNA diseases.

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12 December 2020

Infantile encephalopathy associated with the MELAS A3243G mutation

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12 December 2020

A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine

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12 December 2020

Mitochondrial DNA: Radically free of free-radical driven mutations

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12 December 2020

Limitations of Allotopic Expression of Mitochondrial Genes in Mammalian Cells

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12 December 2020

Nucleotide sequence evidence for rapid genotypic shifts in the bovine mitochondrial DNA D-loop

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12 December 2020

Distal weakness with respiratory insufficiency caused by the m.8344A > G "MERRF" mutation

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12 December 2020

Mitochondrial threshold effects.

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12 December 2020

Correcting human mitochondrial mutations with targeted RNA import

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12 December 2020

The incidence of inherited metabolic disorders in the West Midlands, UK.

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12 December 2020

A simple cipher governs DNA recognition by TAL effectors

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12 December 2020

The genetics of mitochondrial disease.

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12 December 2020

Allotopic expression of mitochondrial-encoded genes in mammals: achieved goal, undemonstrated mechanism or impossible task?

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12 December 2020

Coupling of phosphorylation to electron and hydrogen transfer by a chemi-osmotic type of mechanism

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12 December 2020

Directed evolution of the TALE N-terminal domain for recognition of all 5' bases.

1 reference

12 December 2020

Spontaneous Kearns-Sayre/chronic external ophthalmoplegia plus syndrome associated with a mitochondrial DNA deletion: a slip-replication model and metabolic therapy

1 reference

12 December 2020

Selection against heteroplasmy explains the evolution of uniparental inheritance of mitochondria

1 reference

12 December 2020

The molecular machinery of Keilin's respiratory chain

1 reference

12 December 2020

The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA

1 reference

12 December 2020

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

1 reference

12 December 2020

Defects in mitochondrial DNA replication and human disease

1 reference

12 December 2020

Oxidative stress induces degradation of mitochondrial DNA

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12 December 2020

New patterns of inheritance in mitochondrial disease

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12 December 2020

Thymidine phosphorylase gene mutations in MNGIE, a human mitochondrial disorder

1 reference

12 December 2020

Familial mitochondrial encephalomyopathy (MERRF): genetic, pathophysiological, and biochemical characterization of a mitochondrial DNA disease

1 reference

12 December 2020

Hungry codons promote frameshifting in human mitochondrial ribosomes

1 reference

12 December 2020

Reversal of a mitochondrial DNA defect in human skeletal muscle.

1 reference

12 December 2020

Identification of a novel mutation in the mtDNA ND5 gene associated with MELAS.

1 reference

12 December 2020

Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome

1 reference

12 December 2020

The respiratory chain and oxidative phosphorylation

1 reference

12 December 2020

Oxidative damage in mitochondrial DNA is not extensive

1 reference

12 December 2020

Manipulation of mtDNA heteroplasmy in all striated muscles of newborn mice by AAV9-mediated delivery of a mitochondria-targeted restriction endonuclease.

1 reference

12 December 2020

Mitochondrial DNA sequence characteristics modulate the size of the genetic bottleneck

1 reference

12 December 2020

Specific elimination of mutant mitochondrial genomes in patient-derived cells by mitoTALENs

1 reference

12 December 2020

Germline mitochondrial DNA mutations aggravate ageing and can impair brain development

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12 December 2020

Functional human mitochondrial DNA polymerase gamma forms a heterotrimer

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12 December 2020

Coupled leading- and lagging-strand synthesis of mammalian mitochondrial DNA.

1 reference

12 December 2020

Strong purifying selection in transmission of mammalian mitochondrial DNA

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12 December 2020

The adenine nucleotide translocase: a central component of the mitochondrial permeability transition pore and key player in cell death.

1 reference

12 December 2020

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

1 reference

12 December 2020

Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies

1 reference

12 December 2020

A homoplasmic mitochondrial transfer ribonucleic acid mutation as a cause of maternally inherited hypertrophic cardiomyopathy.

1 reference

12 December 2020

Mitochondrial mutations may increase oxidative stress: implications for carcinogenesis and aging?

1 reference

12 December 2020

Requirements for double-strand cleavage by chimeric restriction enzymes with zinc finger DNA-recognition domains

1 reference

12 December 2020

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

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12 December 2020

Melas: an original case and clinical criteria for diagnosis

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12 December 2020

Structure-based redesign of the dimerization interface reduces the toxicity of zinc-finger nucleases

1 reference

12 December 2020

Unconventional decoding of the AUA codon as methionine by mitochondrial tRNAMet with the anticodon f5CAU as revealed with a mitochondrial in vitro translation system

1 reference

12 December 2020

The maintenance of mitochondrial DNA integrity--critical analysis and update

1 reference

12 December 2020

The clinical characteristics of pedigrees of Leber's hereditary optic neuropathy with the 11778 mutation

1 reference

12 December 2020

Mouse L cell mitochondrial DNA molecules are selected randomly for replication throughout the cell cycle

1 reference

12 December 2020

Why do we still have a maternally inherited mitochondrial DNA? Insights from evolutionary medicine

1 reference

12 December 2020

Mitochondrial gene segregation in mammals: is the bottleneck always narrow?

1 reference

12 December 2020

The mitochondrial DNA genetic bottleneck results from replication of a subpopulation of genomes

1 reference

12 December 2020

POLG mutations associated with Alpers' syndrome and mitochondrial DNA depletion

1 reference

12 December 2020

The inheritance of mitochondrial DNA heteroplasmy: random drift, selection or both?

1 reference

12 December 2020

Manipulating mitochondrial DNA heteroplasmy by a mitochondrially targeted restriction endonuclease

1 reference

12 December 2020

Mitochondrial DNA depletion and dGK gene mutations

1 reference

12 December 2020

Mitochondrial endogenous oxidative damage has been overestimated

1 reference

12 December 2020

Genome editing with Cas9 in adult mice corrects a disease mutation and phenotype.

1 reference

12 December 2020

PGC-1alpha/beta upregulation is associated with improved oxidative phosphorylation in cells harboring nonsense mtDNA mutations

1 reference

12 December 2020

Regulation of cell death: the calcium-apoptosis link

1 reference

12 December 2020

Mitochondrially targeted ZFNs for selective degradation of pathogenic mitochondrial genomes bearing large-scale deletions or point mutations

1 reference

12 December 2020

Mutations of the mitochondrial ND1 gene as a cause of MELAS.

1 reference

12 December 2020

Rescue of a mitochondrial deficiency causing Leber Hereditary Optic Neuropathy

1 reference

12 December 2020

Pathogenic mitochondrial DNA mutations are common in the general population

1 reference

12 December 2020

Number matters: control of mammalian mitochondrial DNA copy number

1 reference

12 December 2020

Measurement of oxidatively induced base lesions in liver from Wistar rats of different ages

1 reference

12 December 2020

Occurrence of a multiple sclerosis-like illness in women who have a Leber's hereditary optic neuropathy mitochondrial DNA mutation

1 reference

12 December 2020

PGD and heteroplasmic mitochondrial DNA point mutations: a systematic review estimating the chance of healthy offspring

1 reference

12 December 2020

POLG1 mutations associated with progressive encephalopathy in childhood

1 reference

12 December 2020

Human diseases with impaired mitochondrial protein synthesis

1 reference

12 December 2020

Mitochondrial encephalomyopathies

1 reference

12 December 2020

Towards germline gene therapy of inherited mitochondrial diseases

1 reference

12 December 2020

Somatic mitochondrial DNA mutations in mammalian aging

1 reference

12 December 2020

Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy

1 reference

12 December 2020

Mitochondrial proton and electron leaks

1 reference

12 December 2020

Gene shifting: a novel therapy for mitochondrial myopathy.

1 reference

12 December 2020

Next generation sequencing to characterize mitochondrial genomic DNA heteroplasmy

1 reference

12 December 2020

Development and applications of CRISPR-Cas9 for genome engineering

1 reference

12 December 2020

Preimplantation genetic diagnosis in mitochondrial DNA disorders: challenge and success

1 reference

12 December 2020

Protein engineering: The fate of fingers

1 reference

12 December 2020

Random genetic drift in the female germline explains the rapid segregation of mammalian mitochondrial DNA.

1 reference

12 December 2020

Mitochondrial DNA heteroplasmy in the emerging field of massively parallel sequencing

1 reference

12 December 2020

Repair of 8-oxoG is slower in endogenous nuclear genes than in mitochondrial DNA and is without strand bias.

1 reference

12 December 2020

Multiple neonatal deaths due to a homoplasmic mitochondrial DNA mutation

1 reference

12 December 2020

Mitochondrial DNA mutations and human disease

1 reference

12 December 2020

Mitochondrial DNA genetics and the heteroplasmy conundrum in evolution and disease.

1 reference

12 December 2020

Efficient Mitochondrial Genome Editing by CRISPR/Cas9.

1 reference

12 December 2020

Energetics, epigenetics, mitochondrial genetics

1 reference

12 December 2020

Altering the balance between healthy and mutated mitochondrial DNA.

1 reference

12 December 2020

Mutant mitochondrial thymidine kinase in mitochondrial DNA depletion myopathy

1 reference

12 December 2020

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

1 reference

12 December 2020

Maternal inheritance of human mitochondrial DNA

1 reference

12 December 2020

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

1 reference

12 December 2020

Clinical features associated with the A-->G transition at nucleotide 8344 of mtDNA ("MERRF mutation").

1 reference

12 December 2020

Identifiers

DOI

10.2741/4529

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6986768&resulttype=core&format=json

14 May 2020

PubMed publication ID

27814659

1 reference