Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
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Dual Role of ROS as Signal and Stress Agents: Iron Tips the Balance in favor of Toxic EffectsIron chelation and multiple sclerosisMitochondrial iron-sulfur cluster dysfunction in neurodegenerative diseaseThe Molecular Basis of Iron-induced Oligomerization of Frataxin and the Role of the Ferroxidation Reaction in OligomerizationGeneration and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell modelsOverlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold proteinHighly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells.Molecular insights into frataxin-mediated iron supply for heme biosynthesis in Bacillus subtilisMagmas functions as a ROS regulator and provides cytoprotection against oxidative stress-mediated damagesA new cellular model to follow Friedreich's ataxia development in a time-resolved way.The Structure of the Complex between Yeast Frataxin and Ferrochelatase: CHARACTERIZATION AND PRE-STEADY STATE REACTION OF FERROUS IRON DELIVERY AND HEME SYNTHESIS.Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stressFriedreich ataxia: neuropathology revised.Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarlyHigh-throughput immunoassay for the biochemical diagnosis of Friedreich ataxia in dried blood spots and whole blood.Human Mitochondrial Ferredoxin 1 (FDX1) and Ferredoxin 2 (FDX2) Both Bind Cysteine Desulfurase and Donate Electrons for Iron-Sulfur Cluster Biosynthesis.E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia.Biochemistry of cardiomyopathy in the mitochondrial disease Friedreich's ataxia.Deferiprone for the treatment of Friedreich's ataxia.Review: iron metabolism and the role of iron in neurodegenerative disorders.Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas.Live longer on MARS: a yeast paradigm of mitochondrial adaptive ROS signaling in aging.Oxidative stress in inherited mitochondrial diseases.Hepcidin and its potential clinical utility.Selected missense mutations impair frataxin processing in Friedreich ataxiaAn antioxidant nanozyme that uncovers the cytoprotective potential of vanadia nanowires.Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly MachineryMitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients.Sustained FXN expression in dorsal root ganglia from a nonreplicative genomic HSV-1 vector.Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators.Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers.Protein networks in the maturation of human iron-sulfur proteins.Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia.ISCU(M108I) and ISCU(D39V) Differ from Wild-Type ISCU in Their Failure To Form Cysteine Desulfurase Complexes Containing Both Frataxin and Ferredoxin.Mechanisms of iron and copper-frataxin interactions.Frataxin silencing alters microtubule stability in motor neurons: implications for Friedreich's ataxia.Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly.No effects without causes: the Iron Dysregulation and Dormant Microbes hypothesis for chronic, inflammatory diseases.Drosophila melanogaster Models of Friedreich's Ataxia.
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P2860
Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
@zh
2012年學術文章
@zh-hant
name
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@ast
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@en
type
label
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@ast
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@en
prefLabel
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@ast
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@en
P2860
P1476
Iron-sulfur cluster synthesis, ...... e stress in Friedreich ataxia.
@en
P2093
Grazia Isaya
Rachael A Vaubel
P2860
P356
10.1016/J.MCN.2012.08.003
P577
2012-08-11T00:00:00Z