about
Antioxidants and other pharmacological treatments for Friedreich ataxiaAntioxidants and other pharmacological treatments for Friedreich ataxiaAntioxidants and other pharmacological treatments for Friedreich ataxiaA pool of extramitochondrial frataxin that promotes cell survivalDeficiency in 3'-phosphoglycolate processing in human cells with a hereditary mutation in tyrosyl-DNA phosphodiesterase (TDP1)Friedreich'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 deliveryUnderstanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular modelsTherapeutic developments in Friedreich ataxiaErythropoietin in Friedreich ataxiaOxidative stress and the homeodynamics of iron metabolismFriedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency.Overexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levelsLymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's AtaxiaAutosomal recessive cerebellar ataxias.Proteomic analysis of hearts from frataxin knockout mice: marked rearrangement of energy metabolism, a response to cellular stress and altered expression of proteins involved in cell structure, motility and metabolism.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-binding activity in yeast frataxin entails a trade off with stability in the alpha1/beta1 acidic ridge regionThe structure and function of frataxin.Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblastsPlasma 8-hydroxy-2'-deoxyguanosine Levels in Huntington Disease and Healthy Controls Treated with Coenzyme Q10Friedreich's ataxia: the vicious circle hypothesis revisited.Hepatic mitochondrial dysfunction in Friedreich ataxia.Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxiaMeasuring the rate of progression in Friedreich ataxia: implications for clinical trial design.Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia.Associations between purine metabolites and monoamine neurotransmitters in first-episode psychosis.Exercise capacity and idebenone intervention in children and adolescents with Friedreich ataxiaDevelopment of lipophilic cations as therapies for disorders due to mitochondrial dysfunction.Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.Pharmacological treatments for Friedreich ataxia.Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia.Co-enzyme Q10 and idebenone use in Friedreich's ataxia.Hypothesis: proteasomal dysfunction: a primary event in neurogeneration that leads to nitrative and oxidative stress and subsequent cell death.Stem cells from wildtype and Friedreich's ataxia mice present similar neuroprotective properties in dorsal root ganglia cells.Clinical experience with high-dose idebenone in Friedreich ataxia.The Heart in Friedreich's Ataxia: Basic Findings and Clinical Implications.
P2860
Q24200235-5B835744-D65E-4619-832A-7BFA3E20B026Q24240312-88198231-4067-4707-B983-F051C39E0A82Q24240370-68E880F3-3A29-47DE-9422-74E3F8435C75Q24316486-1C5A58CC-F82A-423A-BE92-745C2CAAADE9Q24557437-7D746B2B-EEFD-4F79-8581-94C719D30FCBQ26830073-2B6082D6-8E48-44BD-B2AE-3A4CCE582996Q26850111-269927B8-9F2D-46F1-9EAD-08EC8576E0A1Q26860523-462F8CC3-F55F-4BAA-BD83-589B1EE6FFC3Q26861733-B0CCFB81-EEBD-4B56-BA3B-F1288DF38004Q26863216-D3B02DE4-4B42-4EFA-B103-E157DB15AB2DQ28082925-0D752BE8-1971-4EDA-A54C-A4D14CD18D4AQ28200651-5EBDB8A0-D497-4292-9D8C-7BEC1C3CBEB5Q28479044-3CEE2F5D-CCA7-437E-A95E-9AE692F4EAE9Q28551371-C642C6BC-8DD1-459A-913A-74090F8B2D31Q31078351-8E54CCA3-4568-483C-8ECF-E68EABFBF765Q33323705-C9CA2C01-33A3-4980-8D52-A81832E6F39EQ33496330-91D630B9-45C7-4FD4-AECA-5F9B103D43FCQ33525513-44BD8BE1-352C-4421-BB26-706F21DA4D64Q33552305-C5F34A7B-0D8B-480D-BFC9-BDE48DC8263FQ33643445-1B70E5C9-EC11-48E6-BADE-5513AF1D1BA7Q33808205-22030AE9-4FBA-4785-B757-53FAD9289409Q33936940-98A47DCB-EB37-4ADF-8C71-7276B3BC34E5Q33970690-745B34F8-F7D5-4029-9529-9305BC2A489DQ34045081-F30A9DF9-626D-4F66-B0B8-1524F081D6E4Q34075023-F29E5BF0-DB5C-443A-9E5F-C282B6B647A4Q34076650-D7F7D35E-4F1E-4309-B392-DA213CB3FAEEQ34143936-547A96A7-AA62-44AC-BE30-0F09ED5564ADQ34197370-E6DC0012-664E-4603-B651-142486DC1DBFQ34317949-C59B201E-6802-4158-92CF-30C523CF02B0Q34351848-025FF54B-9CF0-4AB5-9BF9-277CE9CC064EQ34373154-3AD2D99B-0C31-4BBD-811E-693E6669D51AQ34452604-DF7FB335-9F48-445E-9E7D-B0C992C5697BQ34531992-BB46A20C-0EC6-4911-89A0-026239482C1EQ34538823-73DDD09E-CF08-47C1-B8CD-C05C84FB53B0Q34575719-FCFE1082-FB3F-4A41-B9C7-281EABBB2086Q34654338-2ABC33A1-3292-4A8A-864A-0B5F31B22FDDQ34699662-71889C43-40B2-4564-B352-D0386AAD9CF8Q34720833-41B867A1-0979-4030-A077-042D8D94223EQ34778763-3004EBDE-7A29-4141-978C-FDBC1B502D29Q35052617-44152345-30FE-4CE0-B448-7A33AE6A2637
P2860
description
2000 nî lūn-bûn
@nan
2000 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
Oxidative stress in patients with Friedreich ataxia.
@ast
Oxidative stress in patients with Friedreich ataxia.
@en
type
label
Oxidative stress in patients with Friedreich ataxia.
@ast
Oxidative stress in patients with Friedreich ataxia.
@en
prefLabel
Oxidative stress in patients with Friedreich ataxia.
@ast
Oxidative stress in patients with Friedreich ataxia.
@en
P2093
P356
P1433
P1476
Oxidative stress in patients with Friedreich ataxia
@en
P2093
J Dichgans
M B Bogdanov
P304
P356
10.1212/WNL.55.11.1719
P407
P577
2000-12-01T00:00:00Z