Iron-sulfur protein maturation in human cells: evidence for a function of frataxin (Q24309526)

positive regulation of catalytic activity

GOA release 2020-03-11

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GOA release 2020-03-11

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4

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Roland Lill

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author name string

Oliver Stehling

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Hans-Peter Elsässer

2

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Bernd Brückel

Frataxin gene of Friedreich's ataxia is targeted to mitochondria

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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

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

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Iron metabolism and mitochondrial abnormalities in Friedreich ataxia

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

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

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

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

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

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

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Friedreich's ataxia: treatment within reach

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

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

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

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

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

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

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

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

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

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Iron regulatory proteins in pathobiology.

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

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

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

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

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Crystal structure of human frataxin

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

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

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

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

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

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

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

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

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

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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

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

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

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

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

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

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7 January 2021

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

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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

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7 January 2021