Friedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly Complex - Test2 - elhamkhani - TriplyDB

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

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

http://www.wikidata.org/entity/Q27670432

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Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models Emerging critical roles of Fe-S clusters in DNA replication and repair.Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex The alteration of the C-terminal region of human frataxin distorts its structural dynamics and function.Mitochondrial Cysteine Desulfurase and ISD11 Coexpressed in Escherichia coli Yield Complex Containing Acyl Carrier Protein Overlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold protein Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry.Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.A Yeast/Drosophila Screen to Identify New Compounds Overcoming Frataxin Deficiency Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis.Coming into view: eukaryotic iron chaperones and intracellular iron delivery.Frataxin levels in peripheral tissue in Friedreich ataxia.Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe-S Assembly Complex Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.Human Frataxin Folds Via an Intermediate State. Role of the C-Terminal Region.Selected missense mutations impair frataxin processing in Friedreich ataxia CDKN2A unclassified variants in familial malignant melanoma: combining functional and computational approaches for their assessment.Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery A helix-coil transition induced by the metal ion interaction with a grafted iron-binding site of the CyaY protein family.A dynamic model of the proteins that form the initial iron-sulfur cluster biogenesis machinery in yeast mitochondria.Biophysical characterisation of the recombinant human frataxin precursor.Robust Production, Crystallization, Structure Determination, and Analysis of [Fe-S] Proteins: Uncovering Control of Electron Shuttling and Gating in the Respiratory Metabolism of Molybdopterin Guanine Dinucleotide Enzymes.

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

http://www.wikidata.org/entity/Q27670432

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2011 nî lūn-bûn

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P2860

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

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

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

In vivo maturation of human frataxin

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

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

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

Cysteine desulfurase activity indicates a role for NIFS in metallocluster biosynthesis

Crystal structure of human frataxin

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

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10.1021/BI200666H

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