Cell and animal models of mtDNA biology: progress and prospects (Q34569830)

3 August 2017

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title

Cell and animal models of mtDNA biology: progress and prospects (English)

1 reference

3 August 2017

Shaharyar M Khan

1

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PubMed ID

16899549

3 August 2017

Rafal M Smigrodzki

series ordinal

2

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PubMed ID

16899549

3 August 2017

Russell H Swerdlow

series ordinal

3

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3 August 2017

9 August 2006

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American Journal of Physiology - Cell Physiology

3 August 2017

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3 August 2017

292

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3 August 2017

2

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3 August 2017

C658-69

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Further evidence for mitochondrial dysfunction in progressive supranuclear palsy

3 August 2017

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Splicing-defective mutants of the yeast mitochondrial COXI gene can be corrected by transformation with a hybrid maturase gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Detection of mitochondrial DNA depletion in living human cells using PicoGreen staining.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Protection against ischemic brain injury by protein therapeutics

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Human xenomitochondrial cybrids. Cellular models of mitochondrial complex I deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Dynamics of mitochondria in living cells: shape changes, dislocations, fusion, and fission of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Rapid activation of heat shock factor-1 DNA binding by H2O2 and modulation by glutathione in human neuroblastoma and Alzheimer's disease cybrid cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Functional analysis of lymphoblast and cybrid mitochondria containing the 3460, 11778, or 14484 Leber's hereditary optic neuropathy mitochondrial DNA mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cytoplasmic inheritance of chloramphenicol resistance in mouse tissue culture cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondria dysfunction of Alzheimer's disease cybrids enhances Abeta toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Maternally-inherited Leigh syndrome-related mutations bolster mitochondrial-mediated apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Elevated reactive oxygen species and antioxidant enzyme activities in animal and cellular models of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Interaction among mitochondria, mitogen-activated protein kinases, and nuclear factor-kappaB in cellular models of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cyclosporin A increases resting mitochondrial membrane potential in SY5Y cells and reverses the depressed mitochondrial membrane potential of Alzheimer's disease cybrids.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Determination of normal ranges of mitochondrial respiratory activities by mtDNA transfer from 54 Human subjects to mtDNA-less HeLa cells for identification of the pathogenicities of mutated mtDNAs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Peptide nucleic acid delivery to human mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial transformation of mammalian cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Alzheimer's brains harbor somatic mtDNA control-region mutations that suppress mitochondrial transcription and replication

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Approaches to mitochondrial gene therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mutations in mitochondrial cytochrome c oxidase genes segregate with late-onset Alzheimer disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Human disease-associated mitochondrial mutations fixed in nonhuman primates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Alterations in muscarinic receptor-coupled phosphoinositide hydrolysis and AP-1 activation in Alzheimer's disease cybrid cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Targeting proteins to mitochondria using TAT.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

An established avian fibroblast cell line without mitochondrial DNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Ethidium bromide-induced loss of mitochondrial DNA from primary chicken embryo fibroblasts

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Targeted delivery of DNA to the mitochondrial compartment via import sequence-conjugated peptide nucleic acid

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Structural variety of membrane permeable peptides

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Arginine-rich peptides. An abundant source of membrane-permeable peptides having potential as carriers for intracellular protein delivery

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Use of cytoplasmic hybrid cell lines for elucidating the role of mitochondrial dysfunction in Alzheimer's disease and Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

On auxotrophy for pyrimidines of respiration-deficient chick embryo cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA transmission of the mitochondrial defect in Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Rescue of a mitochondrial deficiency causing Leber Hereditary Optic Neuropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Suppression of a mitochondrial tRNA gene mutation phenotype associated with changes in the nuclear background

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Respiration and growth defects in transmitochondrial cell lines carrying the 11778 mutation associated with Leber's hereditary optic neuropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Tissue-specific distribution of donor mitochondrial DNA in cloned mice produced by somatic cell nuclear transfer.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Suppression of disease phenotypes of adult mito-mice carrying pathogenic mtDNA by bone marrow transplantation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Functional integrity of mitochondrial genomes in human platelets and autopsied brain tissues from elderly patients with Alzheimer's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Inducible expression of a dominant negative DNA polymerase-gamma depletes mitochondrial DNA and produces a rho0 phenotype.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Expanding the functional human mitochondrial DNA database by the establishment of primate xenomitochondrial cybrids

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Alzheimer's disease cybrids replicate beta-amyloid abnormalities through cell death pathways

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Isolation of human cell lines lacking mitochondrial DNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Plant mitochondria actively import DNA via the permeability transition pore complex

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Natural competence of mammalian mitochondria allows the molecular investigation of mitochondrial gene expression.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Different metabolic properties of mitochondrial oxidative phosphorylation in different cell types--important implications for mitochondrial cytopathies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Leber's hereditary optic neuropathy and complex I deficiency in muscle.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cytoplasmic transfer in oocytes: biochemical aspects.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Canine spongiform leukoencephalomyelopathy is associated with a missense mutation in cytochrome b.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

High aggregate burden of somatic mtDNA point mutations in aging and Alzheimer's disease brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA mutation and depletion increase the susceptibility of human cells to apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Consequences of mutations in human DNA polymerase gamma.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON)

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Production of homoplasmic xenomitochondrial mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Aging-dependent large accumulation of point mutations in the human mtDNA control region for replication

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Flock house virus RNA polymerase is a transmembrane protein with amino-terminal sequences sufficient for mitochondrial localization and membrane insertion

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Flock house virus RNA replicates on outer mitochondrial membranes in Drosophila cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Development and characterization of continuous avian cell lines depleted of mitochondrial DNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cardiac disease due to random mitochondrial DNA mutations is prevented by cyclosporin A.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

INTRAMITOCHONDRIAL FIBERS WITH DNA CHARACTERISTICS. I. FIXATION AND ELECTRON STAINING REACTIONS

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

INTRAMITOCHONDRIAL FIBERS WITH DNA CHARACTERISTICS. II. ENZYMATIC AND OTHER HYDROLYTIC TREATMENTS

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Depletion of mitochondrial DNA by ddC in untransformed human cell lines.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Endogenous oxidative stress in sporadic Alzheimer's disease neuronal cybrids reduces viability by increasing apoptosis through pro-death signaling pathways and is mimicked by oxidant exposure of control cybrids

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Activation of p38 and N-acetylcysteine-sensitive c-Jun NH2-terminal kinase signaling cascades is required for induction of apoptosis in Parkinson's disease cybrids

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Altered intracellular signaling and reduced viability of Alzheimer's disease neuronal cybrids is reproduced by beta-amyloid peptide acting through receptor for advanced glycation end products (RAGE).

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Brain-derived growth factor and glial cell line-derived growth factor use distinct intracellular signaling pathways to protect PD cybrids from H2O2-induced neuronal death

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial dysfunction in idiopathic Parkinson disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

The Brucella suis genome reveals fundamental similarities between animal and plant pathogens and symbionts

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

mtDNA mutations increase tumorigenicity in prostate cancer

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Biological markers of oxidative stress in mitochondrial myopathies with progressive external ophthalmoplegia

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Targeting the NAD7 subunit to mitochondria restores a functional complex I and a wild type phenotype in the Nicotiana sylvestris CMS II mutant lacking nad7.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Production of transmitochondrial mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Characterization of human mitochondrial RNase P: novel aspects in tRNA processing.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA modifies cognition in interaction with the nuclear genome and age in mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

TAT-mediated protein transduction and targeted delivery of fusion proteins into mitochondria of breast cancer cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Somatic mitochondrial DNA mutations in cortex and substantia nigra in aging and Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

High frequency of mitochondrial complex I mutations in Parkinson's disease and aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial microheteroplasmy and a theory of aging and age-related disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Localization of HIV RNA in mitochondria of infected cells: potential role in cytopathogenicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial transfer between cells can rescue aerobic respiration

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Manipulating mitochondrial DNA heteroplasmy by a mitochondrially targeted restriction endonuclease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA heteroplasmy in cloned cattle produced by fetal and adult cell cloning

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial dysfunction in cybrid lines expressing mitochondrial genes from patients with progressive supranuclear palsy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cybrids in Alzheimer's disease: a cellular model of the disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondria in sporadic amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Matrilineal inheritance of complex I dysfunction in a multigenerational Parkinson's disease family

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Origin and functional consequences of the complex I defect in Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Role of mitochondria in amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cyclical mitochondrial deltapsiM fluctuations linked to electron transport, F0F1 ATP-synthase and mitochondrial Na+/Ca+2 exchange are reduced in Alzheimer's disease cybrids

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial transcription initiation: promoter structures and RNA polymerases

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Premature ageing in mice expressing defective mitochondrial DNA polymerase

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Differentiated Alzheimer's disease transmitochondrial cybrid cell lines exhibit reduced organelle movement

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial abnormalities in cybrid cell models of sporadic Alzheimer's disease worsen with passage in culture

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Abnormal mitochondrial morphology in sporadic Parkinson's and Alzheimer's disease cybrid cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

MtDNA mutations associated with Leber's hereditary optic neuropathy: studies on cytoplasmic hybrid (cybrid) cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Cytoplasmic transfer of chloramphenicol resistance in human tissue culture cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

The protein import machinery of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Polyethylene-glycol-mediated cybrid formation: High-efficiency techniques and cybrid formation without enucleation

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Transformation of isolated mammalian mitochondria by bacterial conjugation

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

Stable transformation of CHO Cells and human NARP cybrids confers oligomycin resistance (oli(r)) following transfer of a mitochondrial DNA-encoded oli(r) ATPase6 gene to the nuclear genome: a model system for mtDNA gene therapy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPCELL.00224.2006

10.1152/AJPCELL.00224.2006

21 January 2018

Identifiers

DOI

1 reference

3 August 2017

1 reference

PubMed ID

16899549