The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.
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Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegiaIn vivo maturation of human frataxinA pool of extramitochondrial frataxin that promotes cell survivalIron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxiaSalmonella enterica strains lacking the frataxin homolog CyaY show defects in Fe-S cluster metabolism in vivo.Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxiaFriedreich ataxia: an overviewCardiomyopathy 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 deliveryIdebenone and neuroprotection: antioxidant, pro-oxidant, or electron carrier?Transition metals and mitochondrial metabolism in the heartPharmacology of iron transportMitochondrial iron-sulfur cluster dysfunction in neurodegenerative diseaseCrystal structure of human frataxinUnderstanding the binding properties of an unusual metal-binding protein--a study of bacterial frataxinYeast 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 homeostasisIron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia.CCC1 suppresses mitochondrial damage in the yeast model of Friedreich's ataxia by limiting mitochondrial iron accumulation.Friedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency.Infantile onset spinocerebellar ataxia is caused by recessive mutations in mitochondrial proteins Twinkle and TwinkyInduction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth: Otto Warburg revisitedIron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseasesImpaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxiaThe first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxiaGeneration and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell modelsLymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's AtaxiaThe alpha-ketoglutarate dehydrogenase complexNeurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia.Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.Autosomal recessive cerebellar ataxias.Deferiprone targets aconitase: implication for Friedreich's ataxia treatmentThe in vivo mitochondrial two-step maturation of human frataxin.Visual system involvement in patients with Friedreich's ataxia.Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screenAltered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology.PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia.Iron redistribution as a therapeutic strategy for treating diseases of localized iron accumulation.Pyrroloquinoline quinone stimulates mitochondrial biogenesis through cAMP response element-binding protein phosphorylation and increased PGC-1alpha expression
P2860
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P2860
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年学术文章
@wuu
1999年学术文章
@zh-cn
1999年学术文章
@zh-hans
1999年学术文章
@zh-my
1999年学术文章
@zh-sg
1999年學術文章
@yue
1999年學術文章
@zh
1999年學術文章
@zh-hant
name
The Friedreich's ataxia mutati ...... m and inhibitors of apoptosis.
@en
type
label
The Friedreich's ataxia mutati ...... m and inhibitors of apoptosis.
@en
prefLabel
The Friedreich's ataxia mutati ...... m and inhibitors of apoptosis.
@en
P2093
P356
P1476
The Friedreich's ataxia mutati ...... m and inhibitors of apoptosis.
@en
P2093
B Lonnerdal
G Cortopassi
P Cavadini
P304
P356
10.1093/HMG/8.3.425
P577
1999-03-01T00:00:00Z