Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
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Myocardial ischemia in the absence of epicardial coronary artery disease in Friedreich's ataxiaIron and neurodegeneration: from cellular homeostasis to diseaseA pool of extramitochondrial frataxin that promotes cell survivalFriedreich ataxia: an overviewCardiomyopathy in Friedreich ataxia: clinical findings and researchFriedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress HypothesisTherapeutic developments in Friedreich ataxiaTransition metals and mitochondrial metabolism in the heartCrystal structure of human frataxinFriedreich 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 revisitedMuscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disordersAutosomal recessive cerebellar ataxias.Visual system involvement in patients with Friedreich's ataxia.Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology.Flavin adenine dinucleotide rescues the phenotype of frataxin deficiency.Functional characterization of Friedreich ataxia iPS-derived neuronal progenitors and their integration in the adult brain.Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblastsThe neurodegenerative mitochondriopathies.Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Intracellular iron transport and storage: from molecular mechanisms to health implications.Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxiaThe neuro-ophthalmology of mitochondrial diseaseExercise capacity and idebenone intervention in children and adolescents with Friedreich ataxiaMonogenic syndromes of abnormal glucose homeostasis: clinical review and relevance to the understanding of the pathology of insulin resistance and beta cell failure.Genetic analysis of iron citrate toxicity in yeast: implications for mammalian iron homeostasis.Development of lipophilic cations as therapies for disorders due to mitochondrial dysfunction.Molecular basis of Friedreich ataxia.Co-enzyme Q10 and idebenone use in Friedreich's ataxia.Transition of Thalassaemia and Friedreich ataxia from fatal to chronic diseases.Friedreich's ataxia.Clinical experience with high-dose idebenone in Friedreich ataxia.Bioenergetics of the calf muscle in Friedreich ataxia patients measured by 31P-MRS before and after treatment with recombinant human erythropoietin.Cardiomyopathy of Friedreich's ataxia: use of mouse models to understand human disease and guide therapeutic development.The Heart in Friedreich's Ataxia: Basic Findings and Clinical Implications.Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?Frataxin activates mitochondrial energy conversion and oxidative phosphorylation.A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse modelPathways to motor incoordination: the inherited ataxias.
P2860
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P2860
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
1999年论文
@zh
1999年论文
@zh-cn
name
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@ast
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@en
type
label
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@ast
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@en
prefLabel
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@ast
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@en
P2093
P2860
P356
P1476
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
@en
P2093
A H Schapira
D J Taylor
J L Bradley
J M Cooper
P2860
P304
11492-11495
P356
10.1073/PNAS.96.20.11492
P407
P577
1999-09-01T00:00:00Z