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Molecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision MedicineFriedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress HypothesisFunctional and Gait Assessment in Children and Adolescents Affected by Friedreich's Ataxia: A One-Year Longitudinal StudyOxidative stress and the homeodynamics of iron metabolismSensory neuronopathies.Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India.Cerebellar pathology in Friedreich's ataxia: atrophied dentate nuclei with normal iron content.Structural and Functional Magnetic Resonance Imaging of the Cerebellum: Considerations for Assessing Cerebellar Ataxias.Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolismPurkinje Cell Degeneration and Motor Coordination Deficits in a New Mouse Model of Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.Role for the nicotinic cholinergic system in movement disorders; therapeutic implications.Transition of Thalassaemia and Friedreich ataxia from fatal to chronic diseases.Friedreich ataxia: failure of GABA-ergic and glycinergic synaptic transmission in the dentate nucleus.Mechanism of Action of 2-Aminobenzamide HDAC Inhibitors in Reversing Gene Silencing in Friedreich's Ataxia.Phenotypic Screening for Friedreich Ataxia Using Random shRNA Selection.Friedreich ataxia in Norway - an epidemiological, molecular and clinical studyFXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia.Pseudocyst of the auricle in patients with movement disorders: report of two patients with ataxia-associated auricular pseudocysts.New developments and controversies in iron metabolism and iron chelation therapy.Dorsal root ganglia in Friedreich ataxia: satellite cell proliferation and inflammation.Gene Transfer of Brain-derived Neurotrophic Factor (BDNF) Prevents Neurodegeneration Triggered by FXN DeficiencyTwo different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich's ataxia.Friedreich ataxia: metal dysmetabolism in dorsal root ganglia.E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia.Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxiaMitochondrial pathophysiology in Friedreich's ataxia.Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation.Unveiling a common mechanism of apoptosis in β-cells and neurons in Friedreich's ataxia.Autologous stem cell transplant with gene therapy for Friedreich ataxia.Drosophila melanogaster Models of Metal-Related Human Diseases and Metal Toxicity.Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich's Ataxia Transgenic Mice.Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich's ataxia.Reversible Axonal Dystrophy by Calcium Modulation in Frataxin-Deficient Sensory Neurons of YG8R Mice.Urinary, bowel and sexual symptoms in a cohort of patients with Friedreich's ataxia.Deep sequencing of mitochondrial genomes reveals increased mutation load in Friedreich's ataxia.Purkinje cell injury, structural plasticity and fusion in patients with Friedreich's ataxia.Sustained FXN expression in dorsal root ganglia from a nonreplicative genomic HSV-1 vector.iPhemap: an atlas of phenotype to genotype relationships of human iPSC models of neurological diseases.Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia.Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor.
P2860
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on February 2013
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Friedreich ataxia: neuropathology revised.
@en
Friedreich ataxia: neuropathology revised.
@nl
type
label
Friedreich ataxia: neuropathology revised.
@en
Friedreich ataxia: neuropathology revised.
@nl
prefLabel
Friedreich ataxia: neuropathology revised.
@en
Friedreich ataxia: neuropathology revised.
@nl
P2860
P1476
Friedreich ataxia: neuropathology revised.
@en
P2093
Arnulf H Koeppen
Joseph E Mazurkiewicz
P2860
P356
10.1097/NEN.0B013E31827E5762
P577
2013-02-01T00:00:00Z