Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation.
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Dual Role of ROS as Signal and Stress Agents: Iron Tips the Balance in favor of Toxic EffectsIron Homeostasis in Health and DiseaseOxidative stress and the homeodynamics of iron metabolismRole of iron in neurodegenerative diseases.Overlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold proteinLoss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammalsHighly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells.Iron-induced damage in cardiomyopathy: oxidative-dependent and independent mechanisms.A new cellular model to follow Friedreich's ataxia development in a time-resolved way.Protection of scaffold protein Isu from degradation by the Lon protease Pim1 as a component of Fe-S cluster biogenesis regulationProgression of Friedreich ataxia: quantitative characterization over 5 yearsE3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia.Milestones in Friedreich ataxia: more than a century and still learning.Iron and Neurodegeneration: Is Ferritinophagy the Link?Iron quantification with susceptibility.The versatility of the mitochondrial presequence processing machinery: cleavage, quality control and turnover.Translating HDAC inhibitors in Friedreich's ataxia.On the mineral core of ferritin-like proteins: structural and magnetic characterization.Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor.Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia.Nanoscopic X-ray fluorescence imaging and quantification of intracellular key-elements in cryofrozen Friedreich's ataxia fibroblasts.Combined Cerebellar Proton MR Spectroscopy and DWI Study of Patients with Friedreich's Ataxia.Mechanisms, pathophysiological roles and methods for analyzing mitophagy - recent insights.Contribution of Mössbauer spectroscopy to the investigation of Fe/S biogenesis.Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle.No effects without causes: the Iron Dysregulation and Dormant Microbes hypothesis for chronic, inflammatory diseases.Drosophila melanogaster Models of Friedreich's Ataxia.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.A specialized pathway for erythroid iron delivery through lysosomal trafficking of transferrin receptor 2Phenothiazine antioxidants increase mitochondrial biogenesis and frataxin levels in Friedreich's ataxia cellsUnraveling the Role of Heme in Neurodegeneration
P2860
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P2860
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation.
description
2014 nî lūn-bûn
@nan
2014年の論文
@ja
2014年学术文章
@wuu
2014年学术文章
@zh-cn
2014年学术文章
@zh-hans
2014年学术文章
@zh-my
2014年学术文章
@zh-sg
2014年學術文章
@yue
2014年學術文章
@zh
2014年學術文章
@zh-hant
name
Dysregulation of cellular iron ...... tochondrial iron accumulation.
@en
type
label
Dysregulation of cellular iron ...... tochondrial iron accumulation.
@en
prefLabel
Dysregulation of cellular iron ...... tochondrial iron accumulation.
@en
P2860
P356
P1476
Dysregulation of cellular iron ...... itochondrial iron accumulation
@en
P2093
Alain Martelli
P2860
P356
10.3389/FPHAR.2014.00130
P577
2014-06-03T00:00:00Z