Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex
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Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular modelsThe role of mitochondria in cellular iron-sulfur protein biogenesis: mechanisms, connected processes, and diseasesProtein stability and dynamics modulation: the case of human frataxinDyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.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 ProteinOverlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold proteinHuman frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry.Probing the kinetic stabilities of Friedreich's ataxia clinical variants using a solid phase GroEL chaperonin capture platform.Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis.Frataxin levels in peripheral tissue in Friedreich ataxia.Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe-S Assembly ComplexMissense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.Exome sequencing identifies NFS1 deficiency in a novel Fe-S cluster disease, infantile mitochondrial complex II/III deficiency.Brain iron homeostasis: from molecular mechanisms to clinical significance and therapeutic opportunitiesManagement and therapy for cardiomyopathy in Friedreich's ataxia.Human Frataxin Folds Via an Intermediate State. Role of the C-Terminal Region.Selected missense mutations impair frataxin processing in Friedreich ataxiaCompound heterozygous FXN mutations and clinical outcome in friedreich ataxia.Shared Sulfur Mobilization Routes for tRNA Thiolation and Molybdenum Cofactor Biosynthesis in Prokaryotes and Eukaryotes.Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly MachinerySARS-unique fold in the Rousettus bat coronavirus HKU9.A helix-coil transition induced by the metal ion interaction with a grafted iron-binding site of the CyaY protein family.Human mitochondrial ferritin improves respiratory function in yeast mutants deficient in iron-sulfur cluster biogenesis, but is not a functional homologue of yeast frataxin.Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse modelA dynamic model of the proteins that form the initial iron-sulfur cluster biogenesis machinery in yeast mitochondria.Frataxin binds ironBiophysical characterisation of the recombinant human frataxin precursor.
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P2860
Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex
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2011 nî lūn-bûn
@nan
2011 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2011年の論文
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2011年論文
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2011年論文
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2011年論文
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2011年論文
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2011年論文
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2011年论文
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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Structure–Function Analysis of ...... n of the Fe–S Assembly Complex
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P2860
P356
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Structure-function analysis of ...... n of the Fe-S assembly complex
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P2093
Andrew M Winn
Jennifer Bridwell-Rabb
P2860
P304
P356
10.1021/BI200895K
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P577
2011-08-02T00:00:00Z