Peptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia

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Peptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia is …
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scholarly articleQ13442814

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P6179Dimensions Publication ID1091331798
P356DOI10.1038/S41598-017-10320-2
P932PMC publication ID5575096
P698PubMed publication ID28852135

P50authorChuanhui SongQ87461859
P2093author name stringJing Wu
Meng Zhang
Huihui Li
Hongting Zhao
Jiping Chen
Kuanyu Li
Tong Qiao
Shuangying Hao
P2860cites workIron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteinsQ24303938
Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxiaQ24323371
Disabled early recruitment of antioxidant defenses in Friedreich's ataxiaQ74599295
Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary studyQ78180596
Novel cardiolipin therapeutic protects endothelial mitochondria during renal ischemia and mitigates microvascular rarefaction, inflammation, and fibrosisQ87326349
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 ataxiaQ42226636
Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial ironQ43640204
Friedreich's ataxia: idebenone treatment in early stage patientsQ44171040
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 interventionQ45022516
Mitochondria-targeted peptide SS-31 attenuates renal injury via an antioxidant effect in diabetic nephropathyQ46616439
Reversal of Mitochondrial Transhydrogenase Causes Oxidative Stress in Heart Failure.Q46686975
Clinical Experience With Deferiprone Treatment for Friedreich AtaxiaQ47786184
Selective iron chelation in Friedreich ataxia: biologic and clinical implicationsQ48231374
Clinical, biochemical and molecular genetic correlations in Friedreich's ataxiaQ73295495
Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complexQ28115394
Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxiaQ28250989
Activating frataxin expression by repeat-targeted nucleic acidsQ28272901
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansionQ28275699
Targeting mitochondrial cardiolipin and the cytochrome c/cardiolipin complex to promote electron transport and optimize mitochondrial ATP synthesisQ28383442
First-in-class cardiolipin-protective compound as a therapeutic agent to restore mitochondrial bioenergeticsQ28383451
The mitochondrial-targeted compound SS-31 re-energizes ischemic mitochondria by interacting with cardiolipinQ28387020
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 neurotoxicityQ33644322
Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoringQ34200598
Fe2+ binds iron responsive element-RNA, selectively changing protein-binding affinities and regulating mRNA repression and activationQ34274947
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 chemistryQ34434217
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasisQ34499719
Friedreich ataxia: a paradigm for mitochondrial diseasesQ34524337
Pharmacological treatments for Friedreich ataxia.Q34538823
Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trialQ34682814
Friedreich's ataxia: pathology, pathogenesis, and molecular geneticsQ34708637
Mitochondria-targeted antioxidants protect against amyloid-beta toxicity in Alzheimer's disease neuronsQ34772619
Diagnosis and treatment of Friedreich ataxia: a European perspectiveQ34974299
DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA.TTC repeats in Friedreich's ataxiaQ34984370
Mitochondria-targeted peptide accelerates ATP recovery and reduces ischemic kidney injuryQ35012392
Epigenetic therapy for Friedreich ataxiaQ35180607
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxiaQ35579844
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxiaQ35653693
Mitochondria-targeted cytoprotective peptides for ischemia-reperfusion injuryQ36999928
Drug Insight: antioxidant therapy in inherited ataxiasQ37078851
Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's diseaseQ37178581
Global proteomics and pathway analysis of pressure-overload-induced heart failure and its attenuation by mitochondrial-targeted peptidesQ37375934
Evaluating the progression of Friedreich ataxia and its treatmentQ37414038
Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarlyQ37415565
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosisQ38328542
Milestones in Friedreich ataxia: more than a century and still learningQ38348366
Emerging therapies in Friedreich's ataxia.Q38648895
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P4510describes a project that usesImageJQ1659584
P433issue1
P407language of work or nameEnglishQ1860
P921main subjectmitochondrionQ39572
Friedreich ataxiaQ913856
P304page(s)9840
P577publication date2017-08-29
P1433published inScientific ReportsQ2261792
P1476titlePeptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia
P478volume7

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cites work (P2860)
Q89304971Abnormalities of Mitochondrial Dynamics in the Failing Heart: Normalization Following Long-Term Therapy with Elamipretide
Q88136615Cis- and Trans-Modifiers of Repeat Expansions: Blending Model Systems with Human Genetics
Q90314595Identification of Frataxin as a regulator of ferroptosis
Q90567901Mitochondrial Dysfunction Underlies Cardiomyocyte Remodeling in Experimental and Clinical Atrial Fibrillation
Q88197598Protective effects of mitochondrion-targeted peptide SS-31 against hind limb ischemia-reperfusion injury
Q91712186SS-31 Peptide Reverses the Mitochondrial Fragmentation Present in Fibroblasts From Patients With DCMA, a Mitochondrial Cardiomyopathy
Q58700376SS-31 Provides Neuroprotection by Reversing Mitochondrial Dysfunction after Traumatic Brain Injury
Q97587345The cardiolipin-binding peptide elamipretide mitigates fragmentation of cristae networks following cardiac ischemia reperfusion in rats

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