Keywords: Spinocerebellar ataxia type 3; SCA3; Machado-Joseph disease; MJD; Sleep disorders; Sleep apnea;
مقالات ISI (ترجمه نشده)
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Polyglutamine expansion diseases: More than simple repeats
Keywords: Neurodegenerative disease; Polyglutamine protein; Aggregation modulation; Post-translational modification; Molecular partner; Ataxin-3; APR; aggregation-prone region; αB-c; AlphaB-crystallin; AR; androgen receptor; Atx1; ataxin-1; Atx3; ataxin-3; Atn1; a
Activation of NPY-Y2 receptors ameliorates disease pathology in the R6/2 mouse and PC12 cell models of Huntington's disease
Keywords: Aβ; beta-amyloid; ABC; avidin-biotin complex; BBB; blood brain barrier; BDNF; brain derived neurotrophic factor; BSA; bovine serum albumin; CREB; cAMP response element binding protein; cDNA; complementary deoxyribonucleic acid; DAB, 3; 3-diaminobenzidine
Targeting chaperones, heat shock factor-1, and unfolded protein response: Promising therapeutic approaches for neurodegenerative disorders
Keywords: 17-AAG; 17-(allylamino)-17-demethoxygeldanamycin (tanespimycin); 17-DMAG; 17- dimethylaminoethylamino-17-demethoxygeldanamycin (alvespimycin); AAV; adeno-associated virus; Aβ; amyloid-β; AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; APP;
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;
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;
Valproic acid attenuates the suppression of acetyl histone H3 and CREB activity in an inducible cell model of Machado–Joseph disease
Keywords: MJD; Valproic acid; HDAC inhibitor; CREB
Create and preserve: Proteostasis in development and aging is governed by Cdc48/p97/VCP
Keywords: AAA; ATPase associated with diverse cellular activities; AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; AMPK; adenosine monophosphate activated kinase; Cdc48/CDC-48; cell division cycle protein 48; DUB; de-ubiquitylating enzyme; ERAD; endopl
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
DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles
Keywords: ANOVA; analysis of variance; CDK; cyclin-dependent kinase; CREB; cAMP response element-binding protein; DRPLA; dentatorubral-pallidoluysian atrophy; GO; gene ontology; KO; knock-out; MAPK; mitogen-activated protein kinase; MJD; Machado-Joseph disease; N
Toward understanding Machado-Joseph disease
Keywords: ATXN3; ataxin-3; bZIP; basic leucine zipper motif; CBPC; REB binding protein; cDNA; complementary DNA; CHIP; C-terminus of Hsc70 interacting protein; CK2; casein kinase 2; CMV; cytomegalovirus; CNS; central nervous system; CREB; cAMP-response element bind
Thiol-protease oxidation in age-related neuropathology
Keywords: AD; Alzheimer disease; Atg; autophagin; IDE; insulysin; MJD; Machado-Joseph disease; MMP; matrix metalloproteinase; NEP; neprilysin; PD; Parkinson disease; SENP; sentrin-specific protease; SUMO; small-ubiquitin modifier; TIMP-1; tissue inhibitor of meta
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
Mouse Models of Machado-Joseph Disease and Other Polyglutamine Spinocerebellar Ataxias
Keywords: SCA; MJD; mouse model; ataxin; therapy; cleavage fragment;