Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia - Test2 - elhamkhani - TriplyDB

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

http://www.wikidata.org/entity/Q28250989

about

Expression of human frataxin is regulated by transcription factors SRF and TFAP2 In vivo maturation of human frataxin Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia Homozygosity mapping of Portuguese and Japanese forms of ataxia-oculomotor apraxia to 9p13, and evidence for genetic heterogeneity.Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance Mitochondria Vitamin E, antioxidant and nothing more Friedreich ataxia: an overview Survey of human mitochondrial diseases using new genomic/proteomic tools Mitochondrial dynamism and heart disease: changing shape and shaping change Cardiomyopathy in Friedreich ataxia: clinical findings and research Chronochemistry in neurodegeneration Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery Idebenone and neuroprotection: antioxidant, pro-oxidant, or electron carrier?Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models Therapeutic developments in Friedreich ataxia Transition metals and mitochondrial metabolism in the heart Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model Crystal structure of human frataxin Crystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin family Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase.Role of Saccharomyces cerevisiae ISA1 and ISA2 in iron homeostasis Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia.A mitochondrial ferredoxin is essential for biogenesis of cellular iron-sulfur proteins.Suppressors of superoxide dismutase (SOD1) deficiency in Saccharomyces cerevisiae. Identification of proteins predicted to mediate iron-sulfur cluster assembly.CCC1 suppresses mitochondrial damage in the yeast model of Friedreich's ataxia by limiting mitochondrial iron accumulation.Yeast mitochondrial protein, Nfs1p, coordinately regulates iron-sulfur cluster proteins, cellular iron uptake, and iron distribution.Role of the mitochondrial Hsp70s, Ssc1 and Ssq1, in the maturation of Yfh1 Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis.Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae.Adrenodoxin reductase homolog (Arh1p) of yeast mitochondria required for iron homeostasis.YFH1-mediated iron homeostasis is independent of mitochondrial respiration.Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae.An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly.Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis.Mitochondrial Lon protease in human disease and aging: Including an etiologic classification of Lon-related diseases and disorders

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P2860

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

http://www.wikidata.org/entity/Q28250989

description

1997 nî lūn-bûn

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1997 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

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1997 թվականի հոտեմբերին հրատարակված գիտական հոդված

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1997年の論文

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1997年論文

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1997年論文

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1997年論文

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1997年論文

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1997年論文

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1997年论文

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name

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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type

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Nature Genetics

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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A Rötig

http://www.w3.org/2001/XMLSchema#string

D Chretien

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P2860

Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress

Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

Assessment of the mitochondrial respiratory chain.

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria.

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10.1038/NG1097-215

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2

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13899510

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9326946

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