Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Neuroimaging; Cerebellum; FDG-PET; Networks;
مقالات ISI نوع Ataxia نوع 3 Spinocerebellar (ترجمه نشده)
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Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; SCA3; Machado-Joseph disease; MJD; Sleep disorders; Sleep apnea;
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Ataxin-3; Mass spectrometry; Protein interaction; Mitochondria; Atx3; Ataxin-3; CHIP; C-terminus of 70Â kDa heat shock protein; E3s; E3 ubiquitin-protein ligases; HD; Huntington's disease; polyQ; polyglutamine; UPS; ubiquiti
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Arginine vasopressin; Learning and memory; Mirror water maze; Mouse;
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Corticomotoneuron; Transcranial magnetic stimulation;
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Ataxia; Neuronal nicotinic receptors; Nicotine; Olivocerebellar degeneration; Varenicline; 3-AP; 3-acetylpyridine; ANOVA; analysis of variance; SCA3; spinocerebellar ataxia type 3;
Spastic paraparesis as the first manifestation of Machado-Joseph disease: A case report and review of the literature
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Machado-Joseph disease; Spinocerebellar ataxia type 3; Spastic paraparesis; Trinucleotide repeat; Phenotype;
The small heat shock proteins, especially HspB4 and HspB5 are promising protectants in neurodegenerative diseases
Keywords: نوع Ataxia نوع 3 Spinocerebellar; α-Crystallins; Neurodegenerative diseases; Neuroprotection; Gene regulation; Aβ; β-amyloid peptides; AD; Alzheimer's disease; ALS; Amyotrophic lateral sclerosis; AxD; Alexander's disease; CNS; Central nervous system; EAE; experimental autoimmune enceph
Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP/p97 in Drosophila melanogaster
Keywords: نوع Ataxia نوع 3 Spinocerebellar; AAA ATPase; Ataxia; Ataxin-3; Deubiquitinase; Machado-Joseph Disease; Neurodegeneration; Polyglutamine; Spinocerebellar Ataxia Type 3; VCP/p97;
Experimental and Clinical Strategies for Treating Spinocerebellar Ataxia Type 3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; atx-3,; ataxin-3; BDNF,; brain-derived neurotrophic factor; C. elegans,; Caenorhabditis elegans; CBP,; cAMP response element-binding protein CREB-binding protein; CHIP,; C-terminal Hsp70-interacting protein; CK2,; casein kinase 2; DRPLA,; dentatorubral-pa
Exploring the multifaceted roles of heat shock protein B8 (HSPB8) in diseases
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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;
Short CommunicationCancer in Machado-Joseph disease patients-low frequency as a cause of death
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Cancer; causes of death; Machado-Joseph disease; polyglutamine diseases; spinocerebellar ataxia type 3;
Polyglutamine expansion of ataxin-3 alters its degree of ubiquitination and phosphorylation at specific sites
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Ataxin-3; Spinocerebellar ataxia type 3; Mass spectrometry; Neurodegeneration; Ubiquitination; Phosphorylation; DUB; deubiquitinating enzyme;; HD; Huntington's disease; HEK; human embryonic kidney;; LC-MS/MS; liquid chromatography tandem mass spectrometry
A novel nuclear DnaJ protein, DNAJC8, can suppress the formation of spinocerebellar ataxia 3 polyglutamine aggregation in a J-domain independent manner
Keywords: نوع Ataxia نوع 3 Spinocerebellar; PolyQ disease; Spinocerebellar ataxia type 3; DNAJC8; Ataxin-3;
Cervical and ocular vestibular evoked potentials in Machado-Joseph disease: Functional involvement of otolith pathways
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; SCA3; Machado-Joseph disease; MJD; Otolith pathways; Vestibular evoked myogenic potentials; VEMP;
GAPDH binders as potential drugs for the therapy of polyglutamine diseases: Design of a new screening assay
Keywords: نوع Ataxia نوع 3 Spinocerebellar; tTG; tissue transglutaminase; AFM; atomic force microscopy; DARTS; drug affinity responsive target stability; DEP; â(â)deprenyl; DLS; dynamic light scattering; GAPDH; glyceraldehyde-3-phosphate dehydrogenase; GST; glutathione-S-transferase; HD; Huntin
Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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: نوع Ataxia نوع 3 Spinocerebellar; 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;
Spin labeling artery method perfusion MRI study of SPG4 and SCA3/MJD
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado-Joseph disease; Hereditary spastic paraplegia 4; Arterial spin labeling;
Polyglutamine-expanded ataxin-3 impairs long-term depression in Purkinje neurons of SCA3 transgenic mouse by inhibiting HAT and impairing histone acetylation
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Polyglutamine-expanded ataxin-3; Purkinje neuron; Cerebellar long-term depression; Histone acetylation; Histone acetyltransferase
Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: Removal of the CAG containing exon
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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
Different ataxin-3 amyloid aggregates induce intracellular Ca2Â + deregulation by different mechanisms in cerebellar granule cells
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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
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: نوع Ataxia نوع 3 Spinocerebellar; 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;
Sleep disorders in Machado–Joseph disease: A dopamine transporter imaging study
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado–Joseph disease; Sleep disorders; Polysomnography; Dopamine transporter
Chinese patients with spinocerebellar ataxia type 3 presenting with rare clinical symptoms
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Rare clinical features; Heterogeneities
Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Ubiquitin; Post-translational modification; Isopeptide bond; Josephin; Spinocerebellar ataxia type 3; Machado–Joseph disease; Deubiquitinating enzymeATP, adenosine triphosphate; DTT, dithiothreitol; DUB, deubiquitinating enzyme; GST, glutathione-S-transfe
Postural dysfunction in a transgenic mouse model of spinocerebellar ataxia type 3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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
Cognitive and olfactory deficits in Machado–Joseph disease: A dopamine transporter study
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado–Joseph disease; Cognitive deficits; Dopamine transporter; Cognition; Olfaction
The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect
Keywords: نوع Ataxia نوع 3 Spinocerebellar; 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
Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Automated homecage behavior; Genetrap mouse model; Spinocerebellar Ataxia Type 3
Clinical correlates of olfactory dysfunction in spinocerebellar ataxia type 3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado Joseph disease; Olfactory dysfunction
Transcranial sonography findings in spinocerebellar ataxia type 3 (Machado–Joseph disease): A cross-sectional study
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado–Joseph disease; Transcranial sonography; Substantia nigra hyperechogenicity; Brain ultrasound
HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Ataxin-3; Polyglutamine-expanded ataxin-3; SCA3 transgenic mice; Cerebellum; Histone deacetylase
p53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Polyglutamine-expanded ataxin-3; Cerebellum; Pontine nuclei; Bax; p53
Caring for Machado–Joseph disease: Current understanding and how to help patients
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; SCA3; Machado–Joseph disease; CAG
Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: Conclusions from double-transgenic mouse models
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Ataxin-3; Disease modification; Double-transgenic mice; Mouse model; Polyglutamine disease; Spinocerebellar ataxia type 3
A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Machado–Joseph disease; SCA3; MJD; Polyglutamine; Intranuclear inclusion bodies; Transgenic mouse model; CAG repeat instability; Late onset
ATX-3, CDC-48 and UBXN-5: A new trimolecular complex in Caenorhabditis elegans
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Ataxin-3; Polyglutamine; Machado-Joseph disease; Spinocerebellar ataxia type 3; Ubiquitin-proteasome pathway; Deubiquitylating enzyme; Valosin-containing protein;
The polyglutamine-expanded protein ataxin-3 decreases bcl-2 mRNA stability
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Polyglutamine-expanded ataxin-3; Bcl-2; mRNA stability
Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Ataxin-3; Polyglutamine-expanded ataxin-3; SCA3 transgenic mice; Cerebellum; Microarray analysis
Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Ataxin-3; Polyglutamine-expanded ataxin-3; Apoptosis; Polyglutamine neurodegenerative disorders; Bax; Bcl-xL
Dynamic expression of Hsp27 in the presence of mutant ataxin-3
Keywords: نوع Ataxia نوع 3 Spinocerebellar; Spinocerebellar ataxia type 3; Full-length mutant ataxin-3; Heat shock protein 27; Heat shock response;