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Frataxin: a protein in search for a function

Journal of neurochemistry, 2013-08, Vol.126 (s1), p.43-52

2013

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The molecular genetics of coenzyme Q biosynthesis in health and disease

Biochimie, 2014-05, Vol.100, p.78-87

2014

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Friedreich ataxia: the oxidative stress paradox

Human molecular genetics, 2005-02, Vol.14 (4), p.463-474

2005

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Biallelic RFC1-expansion in a French multicentric sporadic ataxia cohort

Journal of neurology, 2021-09, Vol.268 (9), p.3337-3343

2021

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Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy

Brain (London, England : 1878), 2023-08, Vol.146 (8), p.3470-3483

2023

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Clinico‐Genetic, Imaging and Molecular Delineation of COQ8A‐Ataxia: A Multicenter Study of 59 Patients

Annals of neurology, 2020-08, Vol.88 (2), p.251-263

2020

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A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia

Biochimica et biophysica acta. Molecular cell research, 2024-10, Vol.1871 (7), p.119809, Article 119809

2024

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Mammalian Fe-S cluster biogenesis and its implication in disease

Biochimie, 2014-05, Vol.100, p.48-60

2014

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Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex

PloS one, 2011-01, Vol.6 (1), p.e16199-e16199

2011

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Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

Human molecular genetics, 2005-12, Vol.14 (24), p.3857-3864

2005

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Autosomal Recessive Cerebellar Ataxias: Paving the Way toward Targeted Molecular Therapies

Neuron (Cambridge, Mass.), 2019-02, Vol.101 (4), p.560-583

2019

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Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe4S4 Cluster Assembly

Journal of the American Chemical Society, 2013-01, Vol.135 (2), p.733-740

2013

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Correction of half the cardiomyocytes fully rescue Friedreich ataxia mitochondrial cardiomyopathy through cell-autonomous mechanisms

Human molecular genetics, 2019-04, Vol.28 (8), p.1274-1285

2019

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Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

Human molecular genetics, 2004-05, Vol.13 (10), p.1017-1024

2004

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Omaveloxolone: a groundbreaking milestone as the first FDA-approved drug for Friedreich ataxia

Trends in molecular medicine, 2024-02, Vol.30 (2), p.117-125

2024

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Primary Cultures of Pure Embryonic Dorsal Root Ganglia Sensory Neurons as a New Cellular Model for Friedreich's Ataxia

Methods in molecular biology (Clifton, N.J.), 2020-01, Vol.2056, p.241

2020

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Human Nbp35 Is Essential for both Cytosolic Iron-Sulfur Protein Assembly and Iron Homeostasis

Molecular and Cellular Biology, 2008-09, Vol.28 (17), p.5517-5528

2008

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Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation

Frontiers in pharmacology, 2014-06, Vol.5, p.130-130

2014

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Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

Nature medicine, 2014-05, Vol.20 (5), p.542-547

2014

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Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia

Molecular therapy, 2018-08, Vol.26 (8), p.1940-1952

2018

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Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches

Human molecular genetics, 2010-04, Vol.19 (R1), p.R103-R110

2010

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Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment

Brain (London, England : 1878), 2023-09, Vol.146 (9), p.3836-3850

2023

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Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency

Disease models & mechanisms, 2023-10, Vol.16 (10)

2023

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Red blood cell generation from human induced pluripotent stem cells: perspectives for transfusion medicine

Haematologica (Roma), 2010-10, Vol.95 (10), p.1651-1659

2010

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Animal and cellular models of Friedreich ataxia

Journal of neurochemistry, 2013-08, Vol.126 (s1), p.65-79

2013

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Frataxin: a protein in search for a function

The molecular genetics of coenzyme Q biosynthesis in health and disease

Friedreich ataxia: the oxidative stress paradox

Biallelic RFC1-expansion in a French multicentric sporadic ataxia cohort

Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy

Clinico‐Genetic, Imaging and Molecular Delineation of COQ8A‐Ataxia: A Multicenter Study of 59 Patients

A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia

Mammalian Fe-S cluster biogenesis and its implication in disease

Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex

Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

Autosomal Recessive Cerebellar Ataxias: Paving the Way toward Targeted Molecular Therapies

Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe4S4 Cluster Assembly

Correction of half the cardiomyocytes fully rescue Friedreich ataxia mitochondrial cardiomyopathy through cell-autonomous mechanisms

Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

Omaveloxolone: a groundbreaking milestone as the first FDA-approved drug for Friedreich ataxia

Primary Cultures of Pure Embryonic Dorsal Root Ganglia Sensory Neurons as a New Cellular Model for Friedreich's Ataxia

Human Nbp35 Is Essential for both Cytosolic Iron-Sulfur Protein Assembly and Iron Homeostasis

Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation

Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia

Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches

Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment

Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency

Red blood cell generation from human induced pluripotent stem cells: perspectives for transfusion medicine

Animal and cellular models of Friedreich ataxia

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