Frataxin deficiency and mitochondrial dysfunction (Q46667485)

24 December 2017

title

Frataxin deficiency and mitochondrial dysfunction (English)

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24 December 2017

author name string

Massimo Pandolfo

series ordinal

1

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24 December 2017

publication date

1 November 2002

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24 December 2017

published in

Mitochondrion

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24 December 2017

volume

2

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24 December 2017

issue

1-2

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24 December 2017

page(s)

87-93

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24 December 2017

Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia

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Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

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NF kappa B-independent transcriptional induction of the human manganous superoxide dismutase gene

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Diethyldithiocarbamate treatment up regulates manganese superoxide dismutase gene expression in rat liver

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7 January 2021

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

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7 January 2021

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

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7 January 2021

Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae.

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7 January 2021

Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia

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7 January 2021

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

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7 January 2021

Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes

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7 January 2021

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

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7 January 2021

Clinical and genetic abnormalities in patients with Friedreich's ataxia

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7 January 2021

Increased levels of plasma malondialdehyde in Friedreich ataxia

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7 January 2021

Mitochondrial oxidative stress in mice lacking the glutathione peroxidase-1 gene

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7 January 2021

Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain

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7 January 2021

Superoxide radical and iron modulate aconitase activity in mammalian cells

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7 January 2021

Oxygen toxicity, oxygen radicals, transition metals and disease

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7 January 2021

The reaction of no with superoxide

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7 January 2021

The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly

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7 January 2021

Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells

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7 January 2021

Retinoic acid induces embryonal carcinoma cells to differentiate into neurons and glial cells

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7 January 2021

Superoxide accelerates DNA damage by elevating free-iron levels

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7 January 2021

Transcriptional activation of the human manganese superoxide dismutase gene mediated by tetradecanoylphorbol acetate

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7 January 2021

Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia

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7 January 2021

Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia

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7 January 2021

Peroxynitrite-mediated inactivation of manganese superoxide dismutase involves nitration and oxidation of critical tyrosine residues

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7 January 2021

Overexpression of manganese superoxide dismutase suppresses tumor necrosis factor-induced apoptosis and activation of nuclear transcription factor-kappaB and activated protein-1

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7 January 2021

Friedreich's ataxia: MR findings involving the cervical portion of the spinal cord

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7 January 2021

Control of muscle and neuronal differentiation in a cultured embryonal carcinoma cell line

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7 January 2021

Endogenous superoxide dismutase levels regulate iron-dependent hydroxyl radical formation in Escherichia coli exposed to hydrogen peroxide.

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7 January 2021

Importance of Se-glutathione peroxidase, catalase, and Cu/Zn-SOD for cell survival against oxidative stress

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7 January 2021

The Friedreich ataxia GAA triplet repeat: premutation and normal alleles.

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7 January 2021

Somatic mosaicism for Friedreich's ataxia GAA triplet repeat expansions in the central nervous system.

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7 January 2021

Inhibitory effects of expanded GAA.TTC triplet repeats from intron I of the Friedreich ataxia gene on transcription and replication in vivo.

1 reference

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7 January 2021

Induction of Mn-superoxide dismutase by Tumor Necrosis Factor, Interleukin-1 and Interleukin-6 in human hepatoma cells

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7 January 2021

Molecular basis of Friedreich ataxia

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7 January 2021

Novel inhibitors of cytokine-induced IkappaBalpha phosphorylation and endothelial cell adhesion molecule expression show anti-inflammatory effects in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

1 reference

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7 January 2021

Inhibition of mitochondrial electron transport by peroxynitrite

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7 January 2021

The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle

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7 January 2021

Tumor necrosis factor-alpha selectively induces MnSOD expression via mitochondria-to-nucleus signaling, whereas interleukin-1beta utilizes an alternative pathway

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7 January 2021

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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7 January 2021

Sticky DNA: self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia.

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7 January 2021

Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription

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https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Electrophysiological and histological follow-up study in 15 Friedreich's ataxia patients

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7 January 2021

Oxidative stress in patients with Friedreich ataxia.

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7 January 2021

Depletion of the mitochondrial electron transport abrogates the cytotoxic and gene-inductive effects of TNF.

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7 January 2021

The role of hydroxyl radical as a messenger in the activation of nuclear transcription factor NFKB

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7 January 2021

Oxidative mechanisms in the toxicity of metal ions

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https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Purification and characterization of human plasma glutathione peroxidase: a selenoglycoprotein distinct from the known cellular enzyme

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

A yeast mutant lacking mitochondrial manganese-superoxide dismutase is hypersensitive to oxygen

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Significance of MRI-confirmed atrophy of the cranial spinal cord in Friedreich's ataxia

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Increased serum transferrin receptor concentrations in Friedreich ataxia

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

1 reference

https://api.crossref.org/works/10.1016%2FS1567-7249%2802%2900039-9

7 January 2021

Cellular and subcellular localization of catalase in the heart of transgenic mice

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7 January 2021

10.1016/S1567-7249(02)00039-9

Identifiers

DOI

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24 December 2017

PubMed ID

16120311

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24 December 2017