Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity? (Q33210293)

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6 January 2020

title

Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity? (English)

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6 January 2020

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1

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author name string

Ute Bistrich

2

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6 January 2020

Matthias Schranzhofer

3

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6 January 2020

Joseph P Sarsero

4

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6 January 2020

Ursula Rauen

5

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6 January 2020

Barbara Scheiber-Mojdehkar

6

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6 January 2020

Herbert de Groot

7

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Panos Ioannou

8

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Frank Petrat

9

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language of work or name

English

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publication date

22 December 2004

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Journal of Biological Chemistry

6 January 2020

published in

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volume

280

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issue

8

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page(s)

6701-6708

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

6 January 2020

cites work

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

21 January 2018

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

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

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

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Friedreich ataxia-update on pathogenesis and possible therapies

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Molecular insights into Friedreich's ataxia and antioxidant-based therapies

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Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

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Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

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Antioxidant enzymes in blood of patients with Friedreich's ataxia.

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Recent advances in the molecular pathogenesis of Friedreich ataxia

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Increased iron in the dentate nucleus of patients with Friedrich's ataxia

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Sensitivity of FRDA lymphoblasts to salts of transition metal ions.

21 January 2018

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

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The chelatable iron pool in living cells: a methodically defined quantity

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The labile iron pool: characterization, measurement, and participation in cellular processes(1).

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Selective determination of mitochondrial chelatable iron in viable cells with a new fluorescent sensor

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Cold-induced apoptosis of hepatocytes: mitochondrial permeability transition triggered by nonmitochondrial chelatable iron

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Iron-induced mitochondrial permeability transition in cultured hepatocytes

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Calcium vs. iron-mediated processes in hydrogen peroxide toxicity to L929 cells: effects of glucose

21 January 2018

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A specific mRNA binding factor regulates the iron-dependent stability of cytoplasmic transferrin receptor mRNA.

21 January 2018

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Subcellular distribution of chelatable iron: a laser scanning microscopic study in isolated hepatocytes and liver endothelial cells

21 January 2018

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Mitochondrial Membrane Potential in Living Cells

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

21 January 2018

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21 January 2018

Mitochondrial ferritin.

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The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast

21 January 2018

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Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization

21 January 2018

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Structure, function and clinical significance of transferrin receptors

21 January 2018

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Redox properties of human transferrin bound to its receptor

21 January 2018

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Thyroid hormone levels during a 3-week professional road cycling competition.

21 January 2018

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Iron-dependent regulation of ferritin and transferrin receptor expression by the iron-responsive element binding protein

21 January 2018

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Identification of the iron-responsive element for the translational regulation of human ferritin mRNA

21 January 2018

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21 January 2018

Identifiers

DOI

10.1074/JBC.M408717200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15615730%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

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