scholarly article | Q13442814 |
P6179 | Dimensions Publication ID | 1091331798 |
P356 | DOI | 10.1038/S41598-017-10320-2 |
P932 | PMC publication ID | 5575096 |
P698 | PubMed publication ID | 28852135 |
P50 | author | Chuanhui Song | Q87461859 |
P2093 | author name string | Jing Wu | |
Meng Zhang | |||
Huihui Li | |||
Hongting Zhao | |||
Jiping Chen | |||
Kuanyu Li | |||
Tong Qiao | |||
Shuangying Hao | |||
P2860 | cites work | Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins | Q24303938 |
Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia | Q24323371 | ||
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia | Q74599295 | ||
Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study | Q78180596 | ||
Novel cardiolipin therapeutic protects endothelial mitochondria during renal ischemia and mitigates microvascular rarefaction, inflammation, and fibrosis | Q87326349 | ||
Delivery of the 135 kb human frataxin genomic DNA locus gives rise to different frataxin isoforms. | Q38869173 | ||
miR-886-3p levels are elevated in Friedreich ataxia. | Q39319502 | ||
Defective postreperfusion metabolic recovery directly associates with incident delayed graft function. | Q39759505 | ||
Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. | Q40241487 | ||
Mitochondrial membrane potential monitored by JC-1 dye. | Q41386010 | ||
Deficiency of Parkinson's disease-related gene Fbxo7 is associated with impaired mitochondrial metabolism by PARP activation. | Q42081903 | ||
Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia | Q42226636 | ||
Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron | Q43640204 | ||
Friedreich's ataxia: idebenone treatment in early stage patients | Q44171040 | ||
EMBRACE STEMI study: a Phase 2a trial to evaluate the safety, tolerability, and efficacy of intravenous MTP-131 on reperfusion injury in patients undergoing primary percutaneous coronary intervention | Q45022516 | ||
Mitochondria-targeted peptide SS-31 attenuates renal injury via an antioxidant effect in diabetic nephropathy | Q46616439 | ||
Reversal of Mitochondrial Transhydrogenase Causes Oxidative Stress in Heart Failure. | Q46686975 | ||
Clinical Experience With Deferiprone Treatment for Friedreich Ataxia | Q47786184 | ||
Selective iron chelation in Friedreich ataxia: biologic and clinical implications | Q48231374 | ||
Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia | Q73295495 | ||
Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex | Q28115394 | ||
Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia | Q28250989 | ||
Activating frataxin expression by repeat-targeted nucleic acids | Q28272901 | ||
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
Targeting mitochondrial cardiolipin and the cytochrome c/cardiolipin complex to promote electron transport and optimize mitochondrial ATP synthesis | Q28383442 | ||
First-in-class cardiolipin-protective compound as a therapeutic agent to restore mitochondrial bioenergetics | Q28383451 | ||
The mitochondrial-targeted compound SS-31 re-energizes ischemic mitochondria by interacting with cardiolipin | Q28387020 | ||
Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity? | Q33210293 | ||
Mitochondria targeted peptides protect against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity | Q33644322 | ||
Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring | Q34200598 | ||
Fe2+ binds iron responsive element-RNA, selectively changing protein-binding affinities and regulating mRNA repression and activation | Q34274947 | ||
Epigenetic and neurological effects and safety of high-dose nicotinamide in patients with Friedreich's ataxia: an exploratory, open-label, dose-escalation study. | Q34418695 | ||
Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry | Q34434217 | ||
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis | Q34499719 | ||
Friedreich ataxia: a paradigm for mitochondrial diseases | Q34524337 | ||
Pharmacological treatments for Friedreich ataxia. | Q34538823 | ||
Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial | Q34682814 | ||
Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
Mitochondria-targeted antioxidants protect against amyloid-beta toxicity in Alzheimer's disease neurons | Q34772619 | ||
Diagnosis and treatment of Friedreich ataxia: a European perspective | Q34974299 | ||
DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA.TTC repeats in Friedreich's ataxia | Q34984370 | ||
Mitochondria-targeted peptide accelerates ATP recovery and reduces ischemic kidney injury | Q35012392 | ||
Epigenetic therapy for Friedreich ataxia | Q35180607 | ||
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia | Q35579844 | ||
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia | Q35653693 | ||
Mitochondria-targeted cytoprotective peptides for ischemia-reperfusion injury | Q36999928 | ||
Drug Insight: antioxidant therapy in inherited ataxias | Q37078851 | ||
Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease | Q37178581 | ||
Global proteomics and pathway analysis of pressure-overload-induced heart failure and its attenuation by mitochondrial-targeted peptides | Q37375934 | ||
Evaluating the progression of Friedreich ataxia and its treatment | Q37414038 | ||
Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly | Q37415565 | ||
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis | Q38328542 | ||
Milestones in Friedreich ataxia: more than a century and still learning | Q38348366 | ||
Emerging therapies in Friedreich's ataxia. | Q38648895 | ||
P275 | copyright license | Creative Commons Attribution 4.0 International | Q20007257 |
P6216 | copyright status | copyrighted | Q50423863 |
P4510 | describes a project that uses | ImageJ | Q1659584 |
P433 | issue | 1 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | mitochondrion | Q39572 |
Friedreich ataxia | Q913856 | ||
P304 | page(s) | 9840 | |
P577 | publication date | 2017-08-29 | |
P1433 | published in | Scientific Reports | Q2261792 |
P1476 | title | Peptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia | |
P478 | volume | 7 |
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Q88136615 | Cis- and Trans-Modifiers of Repeat Expansions: Blending Model Systems with Human Genetics |
Q90314595 | Identification of Frataxin as a regulator of ferroptosis |
Q90567901 | Mitochondrial Dysfunction Underlies Cardiomyocyte Remodeling in Experimental and Clinical Atrial Fibrillation |
Q88197598 | Protective effects of mitochondrion-targeted peptide SS-31 against hind limb ischemia-reperfusion injury |
Q91712186 | SS-31 Peptide Reverses the Mitochondrial Fragmentation Present in Fibroblasts From Patients With DCMA, a Mitochondrial Cardiomyopathy |
Q58700376 | SS-31 Provides Neuroprotection by Reversing Mitochondrial Dysfunction after Traumatic Brain Injury |
Q97587345 | The cardiolipin-binding peptide elamipretide mitigates fragmentation of cristae networks following cardiac ischemia reperfusion in rats |
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