Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex (Q27670917)

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English	Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex	scientific article

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8 February 2020

Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex (English)

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8 February 2020

Friedreich ataxia

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David P. Barondeau

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8 February 2020

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Jennifer Bridwell-Rabb

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8 February 2020

Andrew M Winn

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2 August 2011

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8 February 2020

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8 February 2020

7265-7274

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8 February 2020

Friedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly Complex

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3340929

20 March 2017

Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron-sulfur cluster biogenesis

1 reference

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20 March 2017

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

1 reference

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20 March 2017

A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis

1 reference

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20 March 2017

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

1 reference

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20 March 2017

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

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20 March 2017

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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20 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3340929

20 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3340929

20 March 2017

Crystal structure of human frataxin

1 reference

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20 March 2017

Cysteine desulfurase activity indicates a role for NIFS in metallocluster biosynthesis

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20 March 2017

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20 March 2017

Free R value: a novel statistical quantity for assessing the accuracy of crystal structures

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20 March 2017

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20 March 2017

Macromolecular TLS refinement in REFMAC at moderate resolutions

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20 March 2017

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20 March 2017

Calculation of protein extinction coefficients from amino acid sequence data

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20 March 2017

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

1 reference

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20 March 2017

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

1 reference

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20 March 2017

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

1 reference

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20 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3340929

20 March 2017

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

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20 March 2017

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

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20 March 2017

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

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20 March 2017

Clinical and genetic abnormalities in patients with Friedreich's ataxia

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20 March 2017

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

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20 March 2017

A combined nucleic acid and protein analysis in Friedreich ataxia: implications for diagnosis, pathogenesis and clinical trial design

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20 March 2017

Structure, function, and formation of biological iron-sulfur clusters

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7 April 2017

Kinetics of allosteric activation

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29 September 2017

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

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29 September 2017

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

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29 September 2017

A rapid, noninvasive immunoassay for frataxin: utility in assessment of Friedreich ataxia

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29 September 2017

Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches.

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29 September 2017

Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities

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29 September 2017

A high throughput electrochemiluminescence assay for the quantification of frataxin protein levels

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29 September 2017

Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis

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29 September 2017

Solution structure of the iron-sulfur cluster cochaperone HscB and its binding surface for the iron-sulfur assembly scaffold protein IscU

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29 September 2017

Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers

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29 September 2017

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29 September 2017

Interaction of the iron-sulfur cluster assembly protein IscU with the Hsc66/Hsc20 molecular chaperone system of Escherichia coli

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29 September 2017

Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu

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2 June 2018

Catalytic mechanism of alpha-retaining glucosyl transfer by Corynebacterium callunae starch phosphorylase: the role of histidine-334 examined through kinetic characterization of site-directed mutants

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The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia

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

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27 November 2018

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

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Iron-sulfur cluster biosynthesis: role of a semi-conserved histidine

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27 November 2018

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

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27 November 2018

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27 November 2018

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

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27 November 2018

Friedreich's ataxia. Revision of the phenotype according to molecular genetics.

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27 November 2018

IscU as a Scaffold for Iron−Sulfur Cluster Biosynthesis: Sequential Assembly of [2Fe-2S] and [4Fe-4S] Clusters in IscU†

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12 December 2020

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Frataxin, a molecule of mystery: trading stability for function in its iron-binding site

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12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A DIRECT MICRODETERMINATION FOR SULFIDE

1 reference

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12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1021/BI200895K

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8 February 2020

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Europe PubMed Central

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8 February 2020

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8 February 2020

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