Cell biological consequences of mitochondrial NADH: ubiquinone oxidoreductase deficiency.

No abstract is available for this paper.

• Publication year

  2004

• Venue

  Current Neurovascular Research

• Publication date

  Unknown publication date

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.2174/1567202043480224PMID 16181064
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Characterization of the human heart mitochondrial proteome

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• Metallothionein‐I Overexpression Decreases Brain Pathology in Transgenic Mice with Astrocyte‐Targeted Expression of Interleukin‐6

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• Human mitochondrial complex I deficiency: investigating transcriptional responses by microarray.

  2003cited by this paper
• The nuclear encoded subunits of complex I from bovine heart mitochondria.

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• Identification and Characterization of a Common Set of Complex I Assembly Intermediates in Mitochondria from Patients with Complex I Deficiency*

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• Structural Characterization of a Human Cytosolic NMN/NaMN Adenylyltransferase and Implication in Human NAD Biosynthesis* 210

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• Analysis of Steady-state Protein Phosphorylation in Mitochondria Using a Novel Fluorescent Phosphosensor Dye*

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• Mitochondrial disorders: Clinical presentation and diagnostic dilemmas

  2003influential reference
• Intra-mitochondrial Poly(ADP-ribosylation) Contributes to NAD+ Depletion and Cell Death Induced by Oxidative Stress*

  2003cited by this paper
• Mitochondria in cell death: novel targets for neuroprotection and cardioprotection.

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• The cristal membrane of mitochondria is the principal site of oxidative phosphorylation

  2003influential reference
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• Transcription factors c‐Jun/activator protein‐1 and nuclear factor‐kappa B in oxidative stress response in mitochondrial diseases

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• The cutting edge of mitochondrial fusion

  2003cited by this paper
• Reconstructing eukaryotic NAD metabolism.

  2003influential reference
• Leber's Hereditary Optic Neuropathy (LHON) Pathogenic Mutations Induce Mitochondrial-dependent Apoptotic Death in Transmitochondrial Cells Incubated with Galactose Medium*

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• Mitochondria‐targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants

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• Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt −1) in the NDUFS4 gene in Leigh syndrome

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• Pathological Mutations of the Human NDUFS4 Gene of the 18-kDa (AQDQ) Subunit of Complex I Affect the Expression of the Protein and the Assembly and Function of the Complex*

  2003influential reference
• When calcium goes wrong: genetic alterations of a ubiquitous signaling route

  2003cited by this paper
• Analysis of the Subunit Composition of Complex I from Bovine Heart Mitochondria*S

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• Blue Native electrophoresis to study mitochondrial and other protein complexes.

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• New nuclear encoded mitochondrial mutation illustrates pitfalls in prenatal diagnosis by biochemical methods.

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• CIA30 complex I assembly factor: a candidate for human complex I deficiency?

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• Protective effect of metallothionein-III on DNA damage in response to reactive oxygen species.

  2002cited by this paper
• Impact of mutations affecting ND mitochondria-encoded subunits on the activity and assembly of complex I in Chlamydomonas. Implication for the structural organization of the enzyme.

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• Antioxidant agents have a different expression pattern in muscle fibers of patients with mitochondrial diseases

  2002cited by this paper
• Resting oxygen consumption and in vivo ADP are increased in myopathy due to complex I deficiency

  2002influential reference
• The Fas Signaling Pathway: More Than a Paradigm

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• Mutations in human nuclear genes encoding for subunits of mitochondrial respiratory complex I: the NDUFS4 gene.

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• Control of oxygen free radical formation from mitochondrial complex I: roles for protein kinase A and pyruvate dehydrogenase kinase.

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• Triacylglycerol infusion improves exercise endurance in patients with mitochondrial myopathy due to complex I deficiency.

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• Triacylglycerol infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy due to complex I deficiency.

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• Cells Bearing Mutations Causing Leber's Hereditary Optic Neuropathy Are Sensitized to Fas-induced Apoptosis*

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• Species-specific and Mutant MWFE Proteins

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• Metal- and tissue-dependent relationship between metallothionein mRNA and protein.

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• Mitochondrial Genetic Control of Assembly and Function of Complex I in Mammalian Cells

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• Diagnosis of mitochondrial disorders: Clinical and biochemical approach

  2001cited by this paper
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  2001cited by this paper
• Enzymatic activities of Sir2 and chromatin silencing.

  2001cited by this paper
• Human Complex I Defects Can Be Resolved by Monoclonal Antibody Analysis into Distinct Subunit Assembly Patterns*

  2001influential reference
• Mutation in the NDUFS4 gene of complex I abolishes cAMP‐dependent activation of the complex in a child with fatal neurological syndrome

  2001cited by this paper
• Prenatal diagnosis of NADH:ubiquinone oxidoreductase deficiency

  2001cited by this paper
• A nonsense mutation in the NDUFS4 gene encoding the 18 kDa (AQDQ) subunit of complex I abolishes assembly and activity of the complex in a patient with Leigh-like syndrome.

  2001cited by this paper
• Respiratory chain complex I deficiency.

  2001cited by this paper
• Human NADH:Ubiquinone Oxidoreductase

  2001influential reference
• NADH Dehydrogenases: From Basic Science to Biomedicine

  2001cited by this paper
• The genetics and pathology of oxidative phosphorylation

  2001influential reference
• Selective Targeting of a Redox-active Ubiquinone to Mitochondria within Cells

  2001cited by this paper
• New functions of a long-known molecule. Emerging roles of NAD in cellular signaling.

  2000cited by this paper
• Sir2 links chromatin silencing, metabolism, and aging.

  2000cited by this paper
• Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 gene.

  2000cited by this paper
• Drug delivery to mitochondria: the key to mitochondrial medicine.

  2000cited by this paper
• Introduction: Our Changing Views of Mitochondria

  2000cited by this paper
• Isolated complex I deficiency in children: Clinical, biochemical and genetic aspects

  2000influential reference
• Application of the obligate aerobic yeast Yarrowia lipolytica as a eucaryotic model to analyse Leigh syndrome mutations in the complex I core subunits PSST and TYKY.

  2000cited by this paper
• Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I

  1999cited by this paper
• Respiratory chain complex I deficiency

  1999influential reference
• Titrating the Effects of Mitochondrial Complex I Impairment in the Cell Physiology*

  1999cited by this paper
• A calcium signaling defect in the pathogenesis of a mitochondrial DNA inherited oxidative phosphorylation deficiency

  1999influential reference
• Does the Patient Have a Mitochondrial Encephalomyopathy?

  1999cited by this paper
• Division versus Fusion: Dnm1p and Fzo1p Antagonistically Regulate Mitochondrial Shape

  1999cited by this paper
• The machinery of mitochondrial inheritance and behavior.

  1999cited by this paper
• Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy

  1999cited by this paper
• Human complex I deficiency: clinical spectrum and involvement of oxygen free radicals in the pathogenicity of the defect.

  1998cited by this paper
• Involvement of two novel chaperones in the assembly of mitochondrial NADH:Ubiquinone oxidoreductase (complex I).

  1998cited by this paper
• Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit.

  1998influential reference
• Molecular remedy of complex I defects: rotenone-insensitive internal NADH-quinone oxidoreductase of Saccharomyces cerevisiae mitochondria restores the NADH oxidase activity of complex I-deficient mammalian cells.

  1998cited by this paper
• Human Xenomitochondrial Cybrids

  1998cited by this paper
• The first nuclear-encoded complex I mutation in a patient with Leigh syndrome.

  1998influential reference
• The mtDNA‐encoded ND6 subunit of mitochondrial NADH dehydrogenase is essential for the assembly of the membrane arm and the respiratory function of the enzyme

  1998cited by this paper
• Close contacts with the endoplasmic reticulum as determinants of mitochondrial Ca2+ responses.

  1998cited by this paper
• Selective targeting of bioactive compounds to mitochondria.

  1997cited by this paper
• Deficiency of respiratory chain complex I is a common cause of leigh disease

  1996cited by this paper
• Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.

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• The nuclear‐encoded 18 kDa (IP) AQDQ subunit of bovine heart complex I is phosphorylated by the mitochondrial cAMP‐dependent protein kinase

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• Familial Cardiomyopathy with Cataracts and Lactic Acidosis: A Defect in Complex I (NADH-Dehydrogenase) of the Mitochondria Respiratory Chain

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• Leigh syndrome: Clinical features and biochemical and DNA abnormalities

  1996influential reference
• Clinical presentations and laboratory investigations in respiratory chain deficiency

  1996influential reference
• The mitochondrial DNA mutation ND6*14,484C associated with leber hereditary optic neuropathy, leads to deficiency of complex I of the respiratory chain.

  1995cited by this paper
• The gene locus of the proton-translocating NADH: ubiquinone oxidoreductase in Escherichia coli. Organization of the 14 genes and relationship between the derived proteins and subunits of mitochondrial complex I.

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• The NADH:ubiquinone oxidoreductase (complex I) of respiratory chains

  1992influential reference
• An ND-6 mitochondrial DNA mutation associated with Leber hereditary optic neuropathy.

  1992cited by this paper
• Reactive oxygen and DNA damage in mitochondria.

  1992cited by this paper
• Mitochondrial creatine kinase containing crystals, creatine content and mitochondrial creatine kinase activity in chronic progressive external ophthalmoplegia.

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• Irreversible Inhibition of Mitochondrial Complex I by 1‐Methyl‐4‐Phenylpyridinium: Evidence for Free Radical Involvement

  1992cited by this paper
• Reactive oxygen species in living systems: source, biochemistry, and role in human disease.

  1991cited by this paper
• The respiratory-chain NADH dehydrogenase (complex I) of mitochondria.

  1991cited by this paper
• 2,3,7,8, tetrachlorodibenzo-p-dioxin induces oxygen activation associated with cell respiration.

  1989cited by this paper
• The biochemical basis of mitochondrial diseases

  1988cited by this paper
• Two types of mitochondrial crystals in diseased human skeletal muscle fibers

  1988cited by this paper
• Sequence and organization of the human mitochondrial genome

  1981cited by this paper
• A mitochondrial myopathy with a deficiency of respiratory chain NADH-CoQ reductase activity.

  1979cited by this paper
• Hydroperoxide metabolism in mammalian organs.

  1979cited by this paper

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• Mitochondrial respiratory chain dysfunction alters ER sterol sensing and mevalonate pathway activity

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• How many molecules of mitochondrial complex I are in a cell?

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  2018cites this paper
• Evaluating the involvement of metallothionein l in complex l deficiency: an in vitro study

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  2017cites this paper
• Mitochondrial-Targeted Molecular Imaging in Cardiac Disease

  2017cites this paper
• Therapeutic potentials of mesenchymal stem cells on the renal cortex of experimentally induced hypertensive albino rats: Relevant role of Nrf2.

  2017cites this paper
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  2016cites this paper
• Mitochondrial disorders in children: toward development of small‐molecule treatment strategies

  2016cites this paper
• Mitochondrial disease associated with complex I (NADH-CoQ oxidoreductase) deficiency

  2015cites this paper
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• Early Mechanisms of Retinal Degeneration in the harlequin Mouse

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  2014cites this paper
• The mitochondrial complex I of trypanosomatids - an overview of current knowledge

  2014cites this paper
• Mitochondrial complex I activity suppresses inflammation and enhances bone resorption by shifting macrophage-osteoclast polarization.

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• A differential pattern of gene expression in skeletal muscle of tumor‐bearing rats reveals dysregulation of excitation–contraction coupling together with additional muscle alterations

  2014cites this paper
• Diagnosis of mitochondrial myopathies.

  2013cites this paper
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  2013cites this paper
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  2013cites this paper
• Molecular Genetics and Metabolism.

  2013cites this paper
• Nuclear gene defects in mitochondrial disorders.

  2012cites this paper
• Points to Consider in Therapy of Mitochondropathy

  2012cites this paper
• The Mitochondrial Complex I Activity Is Reduced in Cells with Impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function

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  2006cites this paper
• Proteomic analysis of mitochondria-to-nucleus retrograde response in human cancer.

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  2006cites this paper
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  2005cites this paper
• Oxidative stress in the brain: novel cellular targets that govern survival during neurodegenerative disease.

  2005cites this paper
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  2005cites this paper
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  2005cites this paper
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  2004cites this paper
• Navigating novel mechanisms of cellular plasticity with the NAD+ precursor and nutrient nicotinamide.

  2004cites this paper