(Q24309526)

positive regulation of catalytic activity

GOA release 2020-03-11

1 reference

GOA release 2020-03-11

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4

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Roland Lill

5

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author name string

Oliver Stehling

1

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Hans-Peter Elsässer

2

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Bernd Brückel

Frataxin gene of Friedreich's ataxia is targeted to mitochondria

3

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language of work or name

English

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publication date

1 December 2004

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published in

Human Molecular Genetics

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volume

13

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issue

23

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page(s)

3007-15

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cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

The cell's cookbook for iron--sulfur clusters: recipes for fool's gold?

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Iron metabolism and mitochondrial abnormalities in Friedreich ataxia

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Ferrochelatase at the millennium: structures, mechanisms and [2Fe-2S] clusters

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

A sensitive, direct colorimetric assay of serum iron using the chromogen, nitro-PAPS.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Biogenesis of iron-sulfur proteins in eukaryotes: a novel task of mitochondria that is inherited from bacteria

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Overexpression of the hereditary hemochromatosis protein, HFE, in HeLa cells induces an iron-deficient phenotype

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Preparation and properties of succinate: ubiquinone oxidoreductase (complex II)

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Aconitases: a class of metalloproteins highly sensitive to nitric oxide synthesis

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Friedreich ataxia: a paradigm for mitochondrial diseases

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Maturation of cellular Fe-S proteins: an essential function of mitochondria

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Formation of iron-sulfur clusters in bacteria: an emerging field in bioinorganic chemistry.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Friedreich's ataxia: treatment within reach

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Biogenesis of iron-sulfur proteins in eukaryotes: components, mechanism and pathology

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

The mitochondrial membrane potential (deltapsi(m)) in apoptosis; an update

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Duplexes of 21-nucleotide RNAs mediate RNA interference in cultured mammalian cells

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Potent and specific genetic interference by double-stranded RNA in Caenorhabditis elegans

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

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

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Iron-induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Iron regulatory proteins in pathobiology.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Crystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin family

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Subcellular compartmentalization of human Nfu, an iron-sulfur cluster scaffold protein, and its ability to assemble a [4Fe-4S] cluster

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Cytoplasmic protein binds in vitro to a highly conserved sequence in the 5' untranslated region of ferritin heavy- and light-subunit mRNAs

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Mechanism of iron transport to the site of heme synthesis inside yeast mitochondria

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Crystal structure of human frataxin

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Mitochondrial control of iron homeostasis. A genome wide analysis of gene expression in a yeast frataxin-deficient strain

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

The heme synthesis defect of mutants impaired in mitochondrial iron-sulfur protein biogenesis is caused by reversible inhibition of ferrochelatase

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Combinatorial mRNA regulation: iron regulatory proteins and iso-iron-responsive elements (Iso-IREs).

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Monitoring of relative mitochondrial membrane potential in living cells by fluorescence microscopy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1).

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Electroporation for the efficient transfection of mammalian cells with DNA.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

A system for stable expression of short interfering RNAs in mammalian cells

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDH324

7 January 2021