Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia. - Wikidata - wikimedia - TriplyDB

Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

http://www.wikidata.org/entity/Q35653693

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Myocardial ischemia in the absence of epicardial coronary artery disease in Friedreich's ataxia Iron and neurodegeneration: from cellular homeostasis to disease A pool of extramitochondrial frataxin that promotes cell survival Friedreich ataxia: an overview Cardiomyopathy in Friedreich ataxia: clinical findings and research Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis Therapeutic developments in Friedreich ataxia Transition metals and mitochondrial metabolism in the heart Crystal structure of human frataxin Friedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency.Infantile onset spinocerebellar ataxia is caused by recessive mutations in mitochondrial proteins Twinkle and Twinky Induction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth: Otto Warburg revisited Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders Autosomal 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 fibroblasts The 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 ataxia The neuro-ophthalmology of mitochondrial disease Exercise capacity and idebenone intervention in children and adolescents with Friedreich ataxia Monogenic 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 model Pathways to motor incoordination: the inherited ataxias.

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P2860

Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

http://www.wikidata.org/entity/Q35653693

description

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

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type

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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.

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

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Proceedings of the National Academy of Sciences of the United States of America

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.

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

Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue

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

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

Clinical and genetic abnormalities in patients with Friedreich's ataxia

Cytosolic phosphorylation potential

Body fat assessed from total body density and its estimation from skinfold thickness: measurements on 481 men and women aged from 16 to 72 Years

Brain and skeletal muscle bioenergetic failure in familial hypobetalipoproteinaemia

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