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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Cardiomyopathy in Friedreich ataxia: clinical findings and researchFriedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress HypothesisIron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster deliveryUnderstanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular modelsCommon defects of mitochondria and iron in neurodegeneration and diabetes (MIND): a paradigm worth exploringThe role of mitochondria in cellular iron-sulfur protein biogenesis: mechanisms, connected processes, and diseasesTransition metals and mitochondrial metabolism in the heartFriedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly ComplexStructure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly ComplexRole of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients.Oxidative stress and the homeodynamics of iron metabolismOverexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levelsTurning Escherichia coli into a Frataxin-Dependent OrganismNeurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in 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 ProteinMutation in the Fe-S scaffold protein Isu bypasses frataxin deletion.Frataxin-bypassing Isu1: characterization of the bypass activity in cells and mitochondriaISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo.Iron and thiols as two major players in carcinogenesis: friends or foes?Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia.Overlapping 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.Novel frataxin isoforms may contribute to the pathological mechanism of Friedreich ataxiaThe L-cysteine desulfurase NFS1 is localized in the cytosol where it provides the sulfur for molybdenum cofactor biosynthesis in humansFe-S cluster biogenesis in isolated mammalian mitochondria: coordinated use of persulfide sulfur and iron and requirements for GTP, NADH, and ATP.Tangled web of interactions among proteins involved in iron-sulfur cluster assembly as unraveled by NMR, SAXS, chemical crosslinking, and functional studies.Isd11p protein activates the mitochondrial cysteine desulfurase Nfs1p protein.Molecular insights into frataxin-mediated iron supply for heme biosynthesis in Bacillus subtilisTurning Saccharomyces cerevisiae into a Frataxin-Independent Organism.A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse modelBiogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis.Frataxin Is Localized to Both the Chloroplast and Mitochondrion and Is Involved in Chloroplast Fe-S Protein Function in ArabidopsisA Yeast/Drosophila Screen to Identify New Compounds Overcoming Frataxin DeficiencyEffector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis.Cysteine desulfurase Nfs1 and Pim1 protease control levels of Isu, the Fe-S cluster biogenesis scaffold.Protein-mediated assembly of succinate dehydrogenase and its cofactorsThe Human Iron-Sulfur Assembly Complex Catalyzes the Synthesis of [2Fe-2S] Clusters on ISCU2 That Can Be Transferred to Acceptor MoleculesFrataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe-S Assembly Complex
P2860
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P2860
Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex.
description
2011 nî lūn-bûn
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2011 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@ast
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@en
type
label
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@ast
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@en
prefLabel
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@ast
Mammalian frataxin: an essenti ...... iron-sulfur assembly complex.
@en
P2093
P2860
P1433
P1476
Mammalian frataxin: an essenti ...... 1 iron-sulfur assembly complex
@en
P2093
Adeline Page
Alain Martelli
Florent Colin
Laurence Reutenauer
Marie Wattenhofer-Donzé
Stéphane Schmucker
P2860
P304
P356
10.1371/JOURNAL.PONE.0016199
P407
P577
2011-01-26T00:00:00Z