(Q38121942)

29 August 2017

Frataxin: a protein in search for a function (English)

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29 August 2017

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Annalisa Pastore

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29 August 2017

Helene Puccio

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language of work or name

English

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publication date

1 August 2013

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Journal of Neurochemistry

29 August 2017

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29 August 2017

126 Suppl 1

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29 August 2017

43-52

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The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria

29 August 2017

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

21 January 2018

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A structural approach to understanding the iron-binding properties of phylogenetically different frataxins.

21 January 2018

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The factors governing the thermal stability of frataxin orthologues: how to increase a protein's stability

21 January 2018

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Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS

21 January 2018

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Understanding cold denaturation: the case study of Yfh1.

21 January 2018

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The role of hydration in protein stability: comparison of the cold and heat unfolded states of Yfh1.

21 January 2018

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The end of "small science"?

21 January 2018

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

21 January 2018

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Iron-sulfur cluster biosynthesis

21 January 2018

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Human frataxin: iron and ferrochelatase binding surface.

21 January 2018

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Iron binding and oxidation kinetics in frataxin CyaY of Escherichia coli.

21 January 2018

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

21 January 2018

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Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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In vivo maturation of human frataxin

21 January 2018

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Monomeric yeast frataxin is an iron-binding protein

21 January 2018

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Molecular details of the yeast frataxin-Isu1 interaction during mitochondrial Fe-S cluster assembly

21 January 2018

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Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding

21 January 2018

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Dynamics, stability and iron-binding activity of frataxin clinical mutants

21 January 2018

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

21 January 2018

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Crystal structure of human frataxin

21 January 2018

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Frataxin, a conserved mitochondrial protein, in the hydrogenosome of Trichomonas vaginalis

21 January 2018

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A non-essential function for yeast frataxin in iron-sulfur cluster assembly

21 January 2018

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

21 January 2018

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Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly

21 January 2018

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Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

21 January 2018

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Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level

21 January 2018

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Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly

21 January 2018

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

21 January 2018

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Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction

21 January 2018

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Frataxin interacts functionally with mitochondrial electron transport chain proteins

21 January 2018

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Yeast frataxin solution structure, iron binding, and ferrochelatase interaction.

21 January 2018

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Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe-S cluster scaffold protein

21 January 2018

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

21 January 2018

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The role of CyaY in iron sulfur cluster assembly on the E. coli IscU scaffold protein

21 January 2018

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The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.

21 January 2018

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

21 January 2018

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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

21 January 2018

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Iron-sulfur cluster biosynthesis: characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU.

21 January 2018

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Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet

21 January 2018

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

21 January 2018

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A human mitochondrial ferritin encoded by an intronless gene

21 January 2018

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Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models

21 January 2018

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Characterization of iron-sulfur protein assembly in isolated mitochondria. A requirement for ATP, NADH, and reduced iron

21 January 2018

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

21 January 2018

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Structure of frataxin iron cores: an X-ray absorption spectroscopic study

21 January 2018

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Assembly of human frataxin is a mechanism for detoxifying redox-active iron

21 January 2018

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Isd11p protein activates the mitochondrial cysteine desulfurase Nfs1p protein

21 January 2018

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Friedreich ataxia: the clinical picture

21 January 2018

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Yeast Frataxin Sequentially Chaperones and Stores Iron by Coupling Protein Assembly with Iron Oxidation

21 January 2018

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Unbiased cold denaturation: low- and high-temperature unfolding of yeast frataxin under physiological conditions

21 January 2018

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The N-terminus of mature human frataxin is intrinsically unfolded

21 January 2018

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Structural bases for the interaction of frataxin with the central components of iron-sulphur cluster assembly

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

21 January 2018

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Fe-S clusters, fragile sentinels of the cell.

21 January 2018

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Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae

21 January 2018

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Structure of the hydrophilic domain of respiratory complex I from Thermus thermophilus

21 January 2018

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The in vivo mitochondrial two-step maturation of human frataxin.

21 January 2018

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Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex.

21 January 2018

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

21 January 2018

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Overexpression of the yeast frataxin homolog (Yfh1): contrasting effects on iron-sulfur cluster assembly, heme synthesis and resistance to oxidative stress

21 January 2018

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Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones.

21 January 2018

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Structural Basis for Fe–S Cluster Assembly and tRNA Thiolation Mediated by IscS Protein–Protein Interactions

21 January 2018

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Oligomerization Propensity and Flexibility of Yeast Frataxin Studied by X-ray Crystallography and Small-Angle X-ray Scattering

21 January 2018

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Aquisition, mobilization and utilization of cellular iron and heme: endless findings and growing evidence of tight regulation

21 January 2018

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Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex

21 January 2018

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Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins

21 January 2018

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Human iron-sulfur cluster assembly, cellular iron homeostasis, and disease

21 January 2018

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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

21 January 2018

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

21 January 2018

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N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins

21 January 2018

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The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin

21 January 2018

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Solution Structure of the Bacterial Frataxin Ortholog, CyaY

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1111/JNC.12220

1 reference

29 August 2017

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PubMed ID

23859340