Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; FALS, familial amyotrophic lateral sclerosis; SALS, sporadic amyotrophic lateral sclerosis; FTD, frontotemporal dementia; GWAS, genome-wide association study; SNP, single-nucleotide polymorphism; NGS, next generation se
مقالات ISI fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی (ترجمه نشده)
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Hydrogen peroxide-mediated induction of SOD1 gene transcription is independent from Nrf2 in a cellular model of neurodegeneration
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, Amyotrophic Lateral Sclerosis; ARE/EpRE, Antioxidant/Electrophile responsive Element; CEs, Composite Elements; fALS, familial Amyotrophic Lateral Sclerosis; NDs, Neurodegenerative Disorders; OS, Oxidative stress; PD, Parkinson's disease; PBMCs, Perip
Single chain variable fragment antibodies block aggregation and toxicity induced by familial ALS-linked mutant forms of SOD1
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; ELISA, enzyme linked immunosorbent assay; FALS, familial amyotrophic lateral sclerosis; HRP, horseradish peroxidase; mAb, monoclonal antibody; MN, motor neuron; mt, mutant; scFvs, single-chain fragments of variable regi
Ethical considerations in the management of amyotrophic lateral sclerosis
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; C9ORF72, C9 Open reading frame 72; FALS, familial amyotrophic lateral sclerosis; FTD, fronto-temporal dementia; FTLD, fronto-temporal lobar dementia; PEG, percutaneous endoscopic gastrostomy; PIG, per-oral image-guided
Reassessment of motor-behavioural test analyses enables the detection of early disease-onset in a transgenic mouse model of amyotrophic lateral sclerosis
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; ANOVA, analysis of variance; fALS, familial amyotrophic lateral sclerosis; hSOD1, human superoxide dismutase 1; sALS, sporadic amyotrophic lateral sclerosisAmyotrophic lateral sclerosis; hSOD1G93A; Mouse model; Grip str
Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotrophic lateral sclerosis: A longitudinal MRI study
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; FALS, familial amyotrophic lateral sclerosis; SOD1, superoxide dismutase 1; MRI, magnetic resonance imaging; PaGE, paw grip endurance7-Tesla MRI; Amyotrophic lateral sclerosis; G93A-SOD1 mice; Neuronal degeneration; Mus
ALS: Focus on purinergic signalling
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; BBG, Brilliant Blue G; BzATP, 3′-O-(benzoyl-4-benzoyl)-ATP; EAAT2, excitatory amino acid transporter 2; fALS, familial amyotrophic lateral sclerosis; GLT-1, glutamate transporter-1; NDPs, nucleoside di-phosphates; NMPs,
Increased SOD1 association with chromatin, DNA damage, p53 activation, and apoptosis in a cellular model of SOD1-linked ALS
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; SOD1, Cu,Zn-superoxide dismutase; ALS, amyotrophic lateral sclerosis; FALS, familial amyotrophic lateral sclerosis; dGuo, 2′-deoxyguanosine; 8-oxodGuo, 8-oxo-7,8-dihydro-2′-deoxyguanosine; MDA, malondialdehyde; TBA, 2-thiobarbituric acid; HPLC, high-perfo
Pigmented creatine deposits in Amyotrophic Lateral Sclerosis central nervous system tissues identified by synchrotron Fourier Transform Infrared microspectroscopy and X-ray fluorescence spectromicroscopy
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; Amyotrophic Lateral Sclerosis; creatine; pigmented deposits; FTIR; XRF; synchrotronAD, Alzheimer disease; ALS, Amyotrophic Lateral Sclerosis; CNV, common copy number variants; FALS, familial Amyotrophic Lateral Sclerosis; FTIR, Fourier Transform Infrared
Measures of bulbar and spinal motor function, muscle innervation, and mitochondrial function in ALS rats
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; CS, citrate synthase; CO, cytochrome oxidase; EDL, extensor digitorum longus; fALS, familial amyotrophic lateral sclerosis; NMJ, neuromuscular junction; RM ANOVA, repeated measures analysis of variance; sALS, sporadic a
Expression of human Cu, Zn-superoxide dismutase in an insect cell-free system and its structural analysis by MALDI-TOF MS
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; CAT, chloramphenicol acetyltransferase; FALS, familial amyotrophic lateral sclerosis; hSOD1, human Cu, Zn-superoxide dismutase; PDI, protein disulfide isomerase; PMF, peptide mass fingerprinting; Sf21, Spodoptera frugiperda 21Cell-free protein synthesis s
Inflammatory cytokines increase mitochondrial damage in motoneuronal cells expressing mutant SOD1
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; fALS, familial amyotrophic lateral sclerosis; GSH, reduced glutathione; GSSG, oxidized glutathione; IFNγ, interferon γ; mutSOD1, mutant SOD1; sALS, sporadic amyotrophic lateral sclerosis; SOD1, Cu,Zn superoxide dismutas
Time-course and characterization of orolingual motor deficits in B6SJL-Tg(SOD1-G93A)1Gur/J mice
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; familial ALS; G1H; bulbar; tongue; operant behavioral task; grip strengthALS, amyotrophic lateral sclerosis; fALS, familial amyotrophic lateral sclerosis; RM ANOVA, repeated measures analysis of variance; SOD1, superoxide-dismutase 1; SOD1-G93A, B6SJL-Tg(
Zinc Binding Modulates the Entire Folding Free Energy Surface of Human Cu,Zn Superoxide Dismutase
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; AEDANS, 5-[[2-(acetylamino)ethyl]amino] napthalene-1-sulfonic acid; ALS, amyotrophic lateral sclerosis; AS-SOD, C6A/C111S SOD; fALS, familial amyotrophic lateral sclerosis; FRET, Förster resonance energy transfer; Gdn-HCl, guanidine hydrochloride; mAS-SOD
Putative denitrosylase activity of Cu,Zn-superoxide dismutase
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; SOD1, Cu,Zn-superoxide dismutase; FALS, familial amyotrophic lateral sclerosis; O2−, superoxide; GSH, glutathione; GSNO, S-nitrosylated glutathione; GSSG, oxidized glutathione; dimedone, 5,5-dimethyl-1,3-cyclohexanedione; EDTA, ethylenediaminetetraacetic
Differential expression of molecular motors in the motor cortex of sporadic ALS
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; CMT 2A, Charcot–Marie–Tooth motor neuronopathy type 2A; FALS, familial amyotrophic lateral sclerosis; KLPs, kinesin-like proteins; ORF, open reading frame; Real-time RT-PCR, real-time reverse transcription-polymerase ch
Mechanisms of axon degeneration: From development to disease
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; AAD, acute axonal degeneration; APP, Alzheimer precursor protein; CNTF, ciliary neurotrophic factor; FALS, familial amyotrophic lateral sclerosis; FF motoneuron, fast-fatiguable motoneuron; FR motoneuron, fast fatigue-resistant motoneuron; MB, mushroom bo
Therapeutic RNA interference for neurodegenerative diseases: From promise to progress
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; AD, Alzheimer’s disease; APP, amyloid precursor protein; BACE1, β-site APP cleaving enzyme; dsRNA, double-stranded RNA; FALS, familial amyotrophic lateral sclerosis; HD, Huntington’s disease; IR, interferon response; IRG, interferon-responsive genes; miRN
Modification of Cysteine 111 in human Cu,Zn-superoxide dismutase
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; hSOD1, human Cu, Zn-superoxide dismutase; LC-MS, liquid chromatography-mass spectrometry; FALS, familial amyotrophic lateral sclerosis; NEM, N-ethylmaleimide; 4VP, 4-vinylpyridine; DTNB, 5,5′-dithiobis(2-nitrobenzoic acid); DTT, dithiothreitol; 2ME, 2-mer
Variable Metallation of Human Superoxide Dismutase: Atomic Resolution Crystal Structures of Cu–Zn, Zn–Zn and As-isolated Wild-type Enzymes
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; copper–zinc superoxide dismutase; familial amyotrophic lateral sclerosis; SOD1; atomic resolution structures; catalysisSOD1, human Cu–Zn superoxide dismutase; FALS, familial amyotrophic lateral sclerosis; XAFS, X-ray absorption fine structure; MAD, multip
Altered distribution and levels of cathepsinD and cystatins in amyotrophic lateral sclerosis transgenic mice: Possible roles in motor neuron survival
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; nerve cell death; cathepsinD; cystatinB; cystatinC; microglia; ALSALS, amyotrophic lateral sclerosis; CSF, cerebrospinal fluid; EPM1, progressive myoclonus epilepsy type 1; ER, endoplasmic reticulum; FALS, familial amyotrophic lateral sclerosis; GFAP, gli
Ascending neuropathology in the CNS of a mutant SOD1 mouse model of amyotrophic lateral sclerosis
Keywords: fals، اسکلروز جانبی جانبی آمیوتروفی فامیلی; ALS, amyotrophic lateral sclerosis; DC, dendritic cell/s; EAE, experimental autoimmune encephalomyelitis; FALS, familial amyotrophic lateral sclerosis; Gall, Gallyas staining; GFAP, glial fibrillary acidic protein; LM, littermate; LMN, lower motorneuron;