Keywords: Inherited autosomal cerebellar ataxia; Dentate nuclei; Atrophy; Magnetic resonance imaging (MRI); Susceptibility-weighted imaging (SWI); AOA1; Ataxia with Oculomotor Apraxia Type 1; AOA2; Ataxia with Oculomotor Apraxia Type 2; AT; Ataxia-Telangiectasia; A
مقالات ISI (ترجمه نشده)
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Keywords: Spinocerebellar ataxia type 3; SCA3; Machado-Joseph disease; MJD; Sleep disorders; Sleep apnea;
Keywords: Ataxia; Neuronal nicotinic receptors; Nicotine; Olivocerebellar degeneration; Varenicline; 3-AP; 3-acetylpyridine; ANOVA; analysis of variance; SCA3; spinocerebellar ataxia type 3;
Exploring the multifaceted roles of heat shock protein B8 (HSPB8) in diseases
Keywords: Aβ; amyloid β-peptide; ACD; α-crystallin domain; AD; Alzheimer disease; ALS; amyotrophic lateral sclerosis; AR; androgen receptor; BAG3; BCL2 associated athanogene 3; BMP; bone morphogenetic protein; CASA; chaperone-assisted selective autophagy; CHIP;
A «aceitação» em portadores assintomáticos e sintomáticos da doença de Machado-Joseph
Keywords: Doença de Machado-Joseph; SCA3; Aceitação; Autocompaixão; Bem-estar psicológico; Satisfação familiar; Mindfulness; Machado-Joseph Disease; SCA3; Acceptance; Self-Compassion; Psychological Well-Being; Family Satisfaction; Mindfulness;
CAG repeat length does not associate with the rate of cerebellar degeneration in spinocerebellar ataxia type 3
Keywords: Brain connectivity; Fractal dimension; Magnetic resonance imaging; Magnetic resonance spectroscopy; NAA; SCA3;
Cervical and ocular vestibular evoked potentials in Machado-Joseph disease: Functional involvement of otolith pathways
Keywords: Spinocerebellar ataxia type 3; SCA3; Machado-Joseph disease; MJD; Otolith pathways; Vestibular evoked myogenic potentials; VEMP;
Quantification of Ataxin-3 and Ataxin-7 aggregates formed in vivo in Drosophila reveals a threshold of aggregated polyglutamine proteins associated with cellular toxicity
Keywords: Polyglutamine disease; Protein aggregates; SCA3; SCA7; Drosophila melanogaster;
Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3
Keywords: polyQ; polyglutamine; HD; Huntington's disease; HTT; Huntingtin protein; SCA3; spinocerebellar ataxia type 3; UPP; ubiquitin-proteasome pathway; UBL; ubiquitin-like domain; UBA; ubiquitin-associated domain; PLIC-1; integrin-associated protein and cytosk
T1-11 and JMF1907 ameliorate polyglutamine-expanded ataxin-3-induced neurodegeneration, transcriptional dysregulation and ataxic symptom in the SCA3 transgenic mouse
Keywords: SCA3; Polyglutamine-expanded ataxin-3; T1-11; JMF1907; SCA3 transgenic mice; Pontine nuclei; Cerebellum; SCA3; spinocerebellar ataxia type 3; T1-11; N6-(4-hydroxybenzyl) adenosine; JMF1907; N6-(3-indolylethyl)adenosine;
A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD
Keywords: Ataxin-3; Mouse; Knock-in; Knockin; SCA3; MJD; Ataxia; Spinocerebellar; CAG repeats; Serpina3n; Polyglutamine;
Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: Removal of the CAG containing exon
Keywords: SCA3; spinocerebellar ataxia type 3; MJD; Machado-Joseph disease; PolyQ; polyglutamine; ATXN3; ataxin-3; UIMs; ubiquitin interacting motifs; RNAi; RNA interference; AON; antisense oligonucleotide; SNP; single nucleotide polymorphism; DMD; Duchenne muscu
H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse
Keywords: Polyglutamine-expanded ataxin-3; Polyglutamine-expanded ataxin-7; H1152; Rho-kinase inhibitor; SCA3 transgenic mice; Pontine nuclei; SCA3; Spinocerebellar ataxia type 3; ROCK; Rho-kinase; SCA7; Spinocerebellar ataxia type 7;
Different ataxin-3 amyloid aggregates induce intracellular Ca2Â + deregulation by different mechanisms in cerebellar granule cells
Keywords: SCA3; spinocerebellar ataxia type 3; SBMA; spinobulbar muscular atrophy; JD; N-terminal Josephin domain; CGNs; rat cerebellar granule neurons; ThT; Thioflavin T; ANS; 8-anilino-1-naphthalenesulfonic acid; AMPA-R; α-amino-3-hydroxy-5-methyl-4-isoxazolepro
Postural dysfunction in a transgenic mouse model of spinocerebellar ataxia type 3
Keywords: 1C2; expanded polyglutamine; ANOVA; analysis of variance; APAs; anticipatory postural adjustments; BF; biceps femoris; EMG; electromyographic; GA; gastrocnemius; HA; hemagglutinin; P; postnatal day; RMS; root mean square; SCA3; spinocerebellar ataxia type
The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect
Keywords: ALS; Amyotrophic Lateral Sclerosis; ATXN2; Ataxin-2; ATXN3; Ataxin-3; PD; Parkinson's disease; polyQ; polyGlutamine domain; SCA2; Spinocerebellar Ataxia type 2; SCA3; Spinocerebellar Ataxia Type 3; Ataxin-2; Ataxin-3; Spinocerebellar Ataxia type 2; Motor
Caring for Machado–Joseph disease: Current understanding and how to help patients
Keywords: Spinocerebellar ataxia type 3; SCA3; Machado–Joseph disease; CAG
A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats
Keywords: Spinocerebellar ataxia type 3; Machado–Joseph disease; SCA3; MJD; Polyglutamine; Intranuclear inclusion bodies; Transgenic mouse model; CAG repeat instability; Late onset
Intrafamilial variability of Parkinson phenotype in SCAs: Novel cases due to SCA2 and SCA3 expansions
Keywords: Parkinson's disease; Dominant inheritance; SCA2; SCA3; Spinocerebellar ataxia
Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination
Keywords: SCA3; Polyglutamine; Neurodegeneration; Knockout; Deubiquitinating enzyme; DUB; Anxiety
Genetic analysis of SCA2, 3 and 17 in idiopathic Parkinson's disease
Keywords: SCA2; SCA3; SCA17; Parkinsonism; Parkinson's disease
Potassium channel dysfunction and depolarized resting membrane potential in a cell model of SCA3
Keywords: SCA3; Polyglutamine; PC12; Resting membrane potential; Potassium channel; Neuronal dysfunction; Electrophysiological dysfunction