Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia (Q24323371)

GOA release 2020-03-11

response to iron ion

1 reference

GOA release 2020-03-11

1 reference

Kuanyu Li

1

0 references

Edward K Besse

2

0 references

Dung Ha

3

0 references

Gennadiy Kovtunovych

4

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Tracey A Rouault

describes a project that uses

5

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language of work or name

English

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publication date

1 August 2008

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published in

Human Molecular Genetics

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volume

17

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issue

15

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page(s)

2265-73

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ImageJ

1 reference

Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC2465796/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

cites work

A pool of extramitochondrial frataxin that promotes cell survival

1 reference

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Roles of the mammalian cytosolic cysteine desulfurase, ISCS, and scaffold protein, ISCU, in iron-sulfur cluster assembly

19 March 2017

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Iron-sulfur protein maturation in human cells: evidence for a function of frataxin

19 March 2017

1 reference

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Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells

19 March 2017

1 reference

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Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase.

19 March 2017

1 reference

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Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis

19 March 2017

1 reference

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Genetic ablations of iron regulatory proteins 1 and 2 reveal why iron regulatory protein 2 dominates iron homeostasis

19 March 2017

1 reference

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

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates

19 March 2017

1 reference

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

19 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

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

19 March 2017

1 reference

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Mammalian tissue oxygen levels modulate iron-regulatory protein activities in vivo

19 March 2017

1 reference

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Balancing acts: molecular control of mammalian iron metabolism

19 March 2017

1 reference

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Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia

7 April 2017

1 reference

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Antioxidant use in Friedreich ataxia

27 September 2017

1 reference

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Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications

27 September 2017

1 reference

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Small molecules affecting transcription in Friedreich ataxia

27 September 2017

1 reference

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Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments.

27 September 2017

1 reference

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Hemin rescues adrenodoxin, heme a and cytochrome oxidase activity in frataxin-deficient oligodendroglioma cells

27 September 2017

1 reference

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Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxia

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Iron and Friedreich ataxia.

27 September 2017

1 reference

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Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.

27 September 2017

1 reference

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The structure and function of frataxin

27 September 2017

1 reference

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Molecular control of vertebrate iron homeostasis by iron regulatory proteins

27 September 2017

1 reference

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DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA.TTC repeats in Friedreich's ataxia

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

The role of iron regulatory proteins in mammalian iron homeostasis and disease

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells.

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

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

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Iron-dependent oxidation, ubiquitination, and degradation of iron regulatory protein 2: implications for degradation of oxidized proteins

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins.

27 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Selective iron chelation in Friedreich ataxia: biologic and clinical implications.

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Increased IRP1 activity in Friedreich ataxia.

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Ancient repeated DNA elements and the regulation of the human frataxin promoter

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Friedreich ataxia: the oxidative stress paradox

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Oxygen and iron regulation of iron regulatory protein 2.

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Upregulation of expression from the FRDA genomic locus for the therapy of Friedreich ataxia

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron

29 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2465796

The role of the mitochondrion in cellular iron homeostasis

29 November 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/18424449

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1093/HMG/DDN127

1 reference

2140915

2140915

1 reference

1 reference

1 reference