The structure and function of frataxin. - Wikidata - awgldk - TriplyDB

The structure and function of frataxin.

The structure and function of frataxin.

http://www.wikidata.org/entity/Q33808205

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Towards a unifying, systems biology understanding of large-scale cellular death and destruction caused by poorly liganded iron: Parkinson's, Huntington's, Alzheimer's, prions, bactericides, chemical toxicology and others as examples In vivo maturation of human frataxin Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance Multi-copper oxidases and human iron metabolism Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease Structural characterization of metal binding to a cold-adapted frataxin Evolution based on domain combinations: the case of glutaredoxins Structural, Mechanistic and Coordination Chemistry of Relevance to the Biosynthesis of Iron-Sulfur and Related Iron Cofactors Iron-binding activity in yeast frataxin entails a trade off with stability in the alpha1/beta1 acidic ridge region Human frataxin: iron and ferrochelatase binding surface.Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol.The 1.4 A crystal structure of the ArsD arsenic metallochaperone provides insights into its interaction with the ArsA ATPase Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Frataxin and mitochondrial FeS cluster biogenesis.Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxia Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly Molecular details of the yeast frataxin-Isu1 interaction during mitochondrial Fe-S cluster assembly Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.Small molecules affecting transcription in Friedreich ataxia Iron-sulfur cluster biogenesis and human disease Friedreich's ataxia: pathology, pathogenesis, and molecular genetics.Probing the kinetic stabilities of Friedreich's ataxia clinical variants using a solid phase GroEL chaperonin capture platform.Molecular insights into frataxin-mediated iron supply for heme biosynthesis in Bacillus subtilis The long history of iron in the Universe and in health and disease.Iron loading site on the Fe-S cluster assembly scaffold protein is distinct from the active site.NMR assignments of a stable processing intermediate of human frataxin.Iron chaperones for mitochondrial Fe-S cluster biosynthesis and ferritin iron storage Key players and their role during mitochondrial iron-sulfur cluster biosynthesis Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.Synthesis, delivery and regulation of eukaryotic heme and Fe-S cluster cofactors.Mitochondrial localization of human frataxin is necessary but processing is not for rescuing frataxin deficiency in Trypanosoma brucei.Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: THE SUB-COMPLEX FORMED BY THE IRON DONOR, Yfh1 PROTEIN, AND THE SCAFFOLD, Isu1 PROTEIN.Assessment of epidermal nerve fibers: a new diagnostic and predictive tool for peripheral neuropathies.Fe-S cluster assembly pathways in bacteria.Drosophila frataxin: an iron chaperone during cellular Fe-S cluster bioassembly.Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers.Iron uptake and transport in plants: the good, the bad, and the ionome.Metal acquisition and availability in the mitochondria.

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The structure and function of frataxin.

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The structure and function of frataxin.

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Critical Reviews in Biochemistry and Molecular Biology

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The structure and function of frataxin.

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César Millán-Pacheco

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Jeremy D Cook

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Kalyan C Kondapalli

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Krisztina Z Bencze

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Nina Pastor

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Stephen McMahon

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Timothy L Stemmler

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P2860

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

Frataxin interacts functionally with mitochondrial electron transport chain proteins

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

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

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

Salmonella enterica strains lacking the frataxin homolog CyaY show defects in Fe-S cluster metabolism in vivo.

The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure

Chelatases: distort to select?

Atypical Friedreich ataxia caused by compound heterozygosity for a novel missense mutation and the GAA triplet-repeat expansion

Friedreich ataxia: an overview

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