Keywords: فراتاکسین; Methylene blue; Mitochondria; Friedreich's ataxia; Frataxin; Cytoprotection;
مقالات ISI فراتاکسین (ترجمه نشده)
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در صورتی که به ترجمه آماده هر یک از مقالات زیر نیاز داشته باشید، می توانید سفارش دهید تا مترجمان با تجربه این مجموعه در اسرع وقت آن را برای شما ترجمه نمایند.
Keywords: فراتاکسین; Frataxin; Mitochondrial dysfunction; Iron metabolism;
Keywords: فراتاکسین; NMR; nuclear magnetic resonance; MD; molecular dynamics; PCA; principal component analysis; ED; essential dynamics; RMSD; root-mean square deviation; RMSF; root-mean-square fluctuation; CyaY; Frataxin; Iron-sulfur cluster biogenesis; Molecular dynamics; S
Keywords: فراتاکسین; Friedreich's ataxia; Conformational dynamics; Backbone motions; Protein engineering; CPMG; Carr-Purcell-Meiboom-Gill; CTR; C-terminal region; FXN; frataxin; hnNOE; heteronuclear 1H-15N NOE; HSQC; heteronuclear single quantum coherence; Rex; contribu
Keywords: فراتاکسین; Friedreich ataxia; Cardiomyopathy; FRATAXIN; Stem cells;
Keywords: فراتاکسین; FA; Friedreich's ataxia; Fxn; frataxin; HAs; human astrocytes; shRNA; short hairpin RNA; ACM; astrocyte conditioned medium; Friedreich's ataxia; Astrocytes; Cell death; Apoptosis; Neurotoxicity; Neurodegeneration;
Keywords: فراتاکسین; ISC; iron-sulfur clusters; FRDA; Friedreich ataxia; NFS1/Nfs1; human/yeast cysteine desulfurase; ISD11/Isd11; human/yeast NFS1-/Nfs1-binding protein; ISCU/Isu1; human/yeast scaffold protein; MPP; mitochondrial processing peptidase; Friedreich ataxia; Fr
Keywords: فراتاکسین; Friedreich’s ataxia; Neurobehavioral; Ataxia; Frataxin; Automated gait analysis; Peripheral sensitivity; Von Frey; Inverted screen
Activating frataxin expression by single-stranded siRNAs targeting the GAA repeat expansion
Keywords: فراتاکسین; Friedreich's ataxia; Frataxin; Gene activation; ss-siRNAs; Antisense oligonucleotides;
Zinc(II) binding on human wild-type ISCU and Met140 variants modulates NFS1 desulfurase activity
Keywords: فراتاکسین; Cysteine desulfurase; Friedreich's ataxia; Iron-sulfur cluster; ISCU; Zinc; ACP; acyl carrier transfer protein; BLI; BioLayer interferometry; BSA; bovine serum albumin; CD; circular dichroism; DMPD; N,N-dimethyl-p-phenylenediamine; DSF; differential scann
Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis
Keywords: فراتاکسین; Friedreich's ataxia; Quantitative proteomics; Frataxin; Mitochondrial metabolism;
Mitochondrial pore opening and loss of Ca2Â + exchanger NCLX levels occur after frataxin depletion
Keywords: فراتاکسین; Frataxin; NCLX; Mitochondrial permeability transition pore (MPTP); NFAT3; Cyclosporin A;
No changes in heme synthesis in human Friedreich´s ataxia erythroid progenitor cells
Keywords: فراتاکسین; AAS; atomicabsorption spectrophotometry; 5-ALA; 5-aminoluvulinic acid; APP; amyloid beta precursor protein; BSA; bovine serum albumin; ECLIA; electrochemiluminescence immunoassay; EPAS1; hypoxia-inducible factor 2-alpha; Epo; erythropoietin; FAS; ferrous
Erythropoietin and small molecule agonists of the tissue-protective erythropoietin receptor increase FXN expression in neuronal cells in vitro and in Fxn-deficient KIKO mice in vivo
Keywords: فراتاکسین; Friedreich's ataxia; Frataxin; Erythropoietin receptor; EPOR/βc agonist; KIKO;
Identification of two frataxin isoforms in Zea mays: Structural and functional studies
Keywords: فراتاکسین; Frataxin; Mitochondria; Fe-S clusters;
Frataxin expression in reticulocytes of non-splenectomized and splenectomized patients with HbE-β-thalassaemia
Keywords: فراتاکسین; Frataxin; Malondialdehyde; Thalassaemia; Oxidative stress; Iron overload;
Characterization of frataxin gene network in Friedreich's ataxia fibroblasts using the RNA-Seq technique
Keywords: فراتاکسین; Frataxin; Friedreich's ataxia; Interferon-induced apoptosis; Hematopoiesis; RNA sequencing;
Delivery of the 135 kb human frataxin genomic DNA locus gives rise to different frataxin isoforms
Keywords: فراتاکسین; Friedreich's ataxia; Frataxin; Gene therapy; Bacterial artificial chromosomes; HSV-1 amplicons
Evidence for chromosome fragility at the frataxin locus in Friedreich ataxia
Keywords: فراتاکسین; Friedreich ataxia; FXN; Frataxin; Repeat expansion; GAA/TTC-triplet repeat; Chromosome fragility
Analysis of Arabidopsis thaliana atfer4-1, atfh and atfer4-1/atfh mutants uncovers frataxin and ferritin contributions to leaf ionome homeostasis
Keywords: فراتاکسین; Arabidopsis; Ferritin; Frataxin; Ionomics; Iron homeostasis; Mitochondria; Molybdenum
Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells
Keywords: فراتاکسین; Friedreich ataxia; Frataxin; Ubiquitin; Orphan drug development;
Novel aberrant genetic and epigenetic events in Friedreich׳s ataxia
Keywords: فراتاکسین; FRDA; Friedreich׳s ataxia; FXN; frataxin; ISC; iron-sulphur cluster; hPDL; human periodontal ligament; CASP3; active caspase-3; DNMT; DNA methyltransferase; CpG; cytosine phosphate group guanine; TSS; transcription start site; UP; upstream GAA repetiti
Assembly of Fe/S proteins in bacterial systems
Keywords: فراتاکسین; Ferredoxin:; Fdx; glutaredoxin:; Grx; Iron-sulfur; Fe/S assembly; Fe/S transfer; Frataxin; Protein complex; Protein-protein interaction;
Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich׳s ataxia
Keywords: فراتاکسین; FRDA; Friedreich׳s ataxia; ISC; iron-sulfur cluster; IRP; iron regulatory protein; Fer1HCH; ferritin 1 heavy chain homologue; Fer2LCH; ferritin 2 light chain homologue; Fer3HCH; mitochondrial ferritin homologue; dmfrn; drosophila mitoferrin; RNAi; RNA
The role of mitochondria and the CIA machinery in the maturation of cytosolic and nuclear iron–sulfur proteins
Keywords: فراتاکسین; Membrane transport; ABC transporter; Atm1–ABCB7; Iron–sulfur cluster; Iron storage disease; Glutathione; Glutaredoxin; Cysteine desulfurase; Frataxin
Interaction of frataxin, an iron binding protein, with IscU of Fe-S clusters biogenesis pathway and its upregulation in AmpB resistant Leishmania donovani
Keywords: فراتاکسین; [Fe-S] clusters; Leishmania donovani; ISC machinery; Frataxin; Ld-frataxin; Protein-protein interaction;
FXN GAA repeat expansions in amyotrophic lateral sclerosis
Keywords: فراتاکسین; Amyotrophic lateral sclerosis; Frataxin; Friedreich's ataxia; Triplet repeats;
Myelin paucity of the superior cerebellar peduncle in individuals with Friedreich ataxia: an MRI magnetization transfer imaging study
Keywords: فراتاکسین; Friedreich ataxia; Axonopathy; Magnetization transfer ratio; Myelin; Frataxin; Oligodendrocytes;
Imatinib inhibits the expression of SCO2 and FRATAXIN genes that encode mitochondrial proteins in human Bcr-Abl+ leukemia cells
Keywords: فراتاکسین; ALL; acute lymphocytic leukemia; COX; cytochrome oxidase; CML; chronic myelogenous leukemia; CS; citrate synthase; FECH; ferrochelatase; FXN; FRATAXIN; HDCBAP; heme-dependent cytochrome c oxidase biosynthesis and assembly pathway; HMOX; heme oxygenase; IM
Mitochondria-derived organelles in the diplomonad fish parasite Spironucleus vortens
Keywords: فراتاکسین; MDO; mitochondria-derived organelles; TEM; transmission electron microscopy; PBS; phosphate-buffered saline at pH 7.2; TMRE; tetramethyl rhodamine ethyl ester; Hydrogenase; Frataxin; Isu1; Flavoprotein; Hydrogenosomes; Mitosomes;
Therapeutic strategies in Friedreich's Ataxia
Keywords: فراتاکسین; Friedreich's Ataxia; Therapeutic Strategies; Frataxin; 17β-estradiol; Methylene blue
Dysregulation of iron protein expression in the G93A model of amyotrophic lateral sclerosis
Keywords: فراتاکسین; ALS; amyotrophic lateral sclerosis; β2MG; β2-microglobulin; Ct; cycle threshold; DCF; dichlorodihydrofluorescein; DFO; deferoxamine; DCFH-DA; dihydrodichlorofluorescein diacetate; DMT1; divalent metal transporter 1; DMEM; Dulbecco's-modified Eagle's
Autophagy induction extends lifespan and reduces lipid content in response to frataxin silencing in C. elegans
Keywords: فراتاکسین; Aging; Nematode; Mitochondria; Frataxin; p53/cep-1; Autophagy; Fat
The role of the N-terminal tail for the oligomerization, folding and stability of human frataxin
Keywords: فراتاکسین; Frataxin; Helical propensity; Protein stability; Oligomerization; Folding;
The role of mitochondria in cellular iron–sulfur protein biogenesis and iron metabolism
Keywords: فراتاکسین; Metal cofactor; Chaperone; Glutaredoxin; Desulfurase; Ferredoxin; Frataxin
Nitric oxide, nitrosyl iron complexes, ferritin and frataxin: A well equipped team to preserve plant iron homeostasis
Keywords: فراتاکسین; Ferritin; Frataxin; Iron nutrition and metabolism; Iron homeostasis; Nitric oxide; Nitrosyl iron complexes
Structural, mechanistic and coordination chemistry of relevance to the biosynthesis of iron–sulfur and related iron cofactors
Keywords: فراتاکسین; Iron–sulfur cluster; Assembly; Biosynthesis; ISU/IscU; Frataxin; NFS/IscS
Friedreich's ataxia: Pathology, pathogenesis, and molecular genetics
Keywords: فراتاکسین; Cardiomyopathy; Dentate nucleus; Dorsal root ganglion; Frataxin; Friedreich's ataxia; GAA trinucleotide repeats; Iron; Transcription;
Protein carbonylation and metal-catalyzed protein oxidation in a cellular perspective
Keywords: فراتاکسین; Carbonylation; Frataxin; Metal binding; Metal-catalyzed oxidation; Mitochondria; Reactive oxygen species4-HNE, 4-hydroxynonenal; AGE, advanced glycation end products; ALE, advanced lipoxidation end products; CML, N(6)-carboxymethyllysine; DNP, 2,4-dinitro
ReviewFriedreich's ataxia: Past, present and future
Keywords: فراتاکسین; Friedreich's ataxia; Frataxin; GAA; Iron; Fe-S cluster; Oxidative stress;
A high throughput electrochemiluminescence assay for the quantification of frataxin protein levels
Keywords: فراتاکسین; Frataxin; Electrochemiluminescence assay; Protein; Quantification
Of the vulnerability of orphan complex proteins: The case study of the E. coli IscU and IscS proteins
Keywords: فراتاکسین; Enzymatic activity; Fluorescence; Frataxin; Iron sulfur cluster; Structure;
A role for p53 in mitochondrial stress response control of longevity in C. elegans
Keywords: فراتاکسین; Mitochondria stress response; p53/cep-1; Aging; Frataxin; C. elegans;
Yeast frataxin mutants display decreased superoxide dismutase activity crucial to promote protein oxidative damage
Keywords: فراتاکسین; Iron overload; Protein carbonylation; Metal-catalyzed oxidation; Frataxin; Yeast; Superoxide dismutase; Iron toxicity; Friedreich ataxia; Free radicals
Nitric oxide accumulation is required to protect against iron-mediated oxidative stress in frataxin-deficient Arabidopsis plants
Keywords: فراتاکسین; Frataxin; Nitric oxide; Iron; Oxidative stress; Arabidopsis thaliana
Overexpression of the yeast frataxin homolog (Yfh1): Contrasting effects on iron–sulfur cluster assembly, heme synthesis and resistance to oxidative stress
Keywords: فراتاکسین; Frataxin; Iron; Iron–sulfur; Heme; Mitochondria; Oxidative stress
Frataxin deficiency induces Schwann cell inflammation and death
Keywords: فراتاکسین; Friedreich's ataxia (FRDA); Dorsal root ganglia (DRG) neuron; Schwann cells; Inflammatory response; siRNA; Frataxin
The import and function of diatom and plant frataxins in the mitochondrion of Trypanosoma brucei
Keywords: فراتاکسین; Trypanosoma; Mitochondrion; Frataxin; Protein import; Arabidopsis; Diatom
Lateral-flow immunoassay for the frataxin protein in Friedreich’s ataxia patients and carriers
Keywords: فراتاکسین; Frataxin; Friedreich’s ataxia; Diagnostic; Prognostic; Theranostic; Lateral flow immunoassay; Mitochondria
Alpha-lipoic acid prevents mitochondrial damage and neurotoxicity in experimental chemotherapy neuropathy
Keywords: فراتاکسین; Neurotoxicity; Mitochondria; Chemotherapy; Alpha-lipoic acid; Frataxin