Keywords: هانتینگتن جهش یافته; 3-HK; 3-hydroxykynurenine; 3-NP; 3-nitropropionic acid; 6âOHDA; 6-hydroxidopamine; AADC; aromatic amino acid decarboxylase; ACA; anterior cerebral artery; ADP; adenosine-5â²-diphosphate; ALDH; aldehyde dehydrogenases; ALS; amyotrophic lateral sclerosis
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Keywords: هانتینگتن جهش یافته; Ambra1; activating molecule in Beclin1-regulated autophagy 1; Atg; autophagy-related protein; BDNF; brain-derived neurotrophic factor; BNIP3; BCL2/adenovirus E1B 19Â kDa interacting protein 3; CREB; cAMP response element-binding; DIC; dynein intermediate
Keywords: هانتینگتن جهش یافته; AD; Alzheimer's disease; PD; Parkinson's disease; HD; Huntington's disease; CJD; Creutzfeld-Jacob disease; mAb; monoclonal antibody; scFv; single-chain Fv; VH; variable domain of the heavy chain; VL; variable domain of the light chain; VHH; variable dom
Keywords: هانتینگتن جهش یافته; Antioxidant gene therapy; Brain ischemia; Neurodegenerative disorders; Oxidative stress; Virus-mediated gene delivery; ROS; ÎΨm; mitochondrial membrane potential; 3-NP; 3-nitropropionic acid; 6-OHDA; 6-hydroxydopamine; AADC; aromatic-l-amino decarboxyla
Keywords: هانتینگتن جهش یافته; Huntington disease; Platelet; Mitochondria; Adenine nucleotides; Mutant huntingtin; Mitochondrial respiratory chain complexes;
Beta catenin is regulated by its subcellular distribution and mutant huntingtin status in Huntington's disease cell STHdhQ111/HdhQ111
Keywords: هانتینگتن جهش یافته; Beta catenin; Huntington's disease; MicroRNA-214; Mutant huntingtin; STHdhQ111/HdhQ111; BDNF; Brain Derived neurotrophic factor; HD; Huntington's Disease; Htt; Huntingtin (mouse); HYPK; Huntingtin Yeast Partner K; miR-214; MicroRNA-214; snRNA; Small Nucle
In vivo methods for acute modulation of gene expression in the central nervous system
Keywords: هانتینگتن جهش یافته; AAV; adeno-associated viruses; ADV; adenoviruses; ApoE; apolypoprotein E; BBB; blood brain barrier; cap; encapsulation; Cas9; CRISPR associated protein 9; cDNA; coding DNA; CMV; cytomegalovirus; CNS; central nervous system; CNT; carbon nanotubes; CRISPR;
Pharmacological disruption of the MID1/α4 interaction reduces mutant Huntingtin levels in primary neuronal cultures
Keywords: هانتینگتن جهش یافته; HTT; Huntingtin; HD; Huntington's Disease; MID1; Midline 1; PP2A; Protein Phosphatase 2A; mHTT; Mutant Huntingtin; wtHTT; Wild type Huntingtin; S6K; 40S ribosomal S6 kinase; mTOR; Mammalian target of rapamycin; CGN; Cerebellar granule neurons; MAP2; Mic
Characterization of subventricular zone-derived progenitor cells from mild and late symptomatic YAC128 mouse model of Huntington's disease
Keywords: هانتینگتن جهش یافته; YAC128 mice; Mutant huntingtin; Subventricular zone; Neurospheres; BDNF; Calcium handling; Mitochondrial membrane potential;
Aberrant subcellular localization of SQSTM1/p62 contributes to increased vulnerability to proteotoxic stress recovery in Huntington's disease
Keywords: هانتینگتن جهش یافته; ATF4; activating transcription factor 4; BSA; bovine serum albumin; CHX; cycloheximide; CQ; hydroxychloroquine; HD; Huntington's disease; HDAC6; histone deacetylase 6; Htt; huntingtin; mHtt; mutant huntingtin; Lamp1; lysosomal associated membrane protein
Activation of NPY-Y2 receptors ameliorates disease pathology in the R6/2 mouse and PC12 cell models of Huntington's disease
Keywords: هانتینگتن جهش یافته; 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
Mitochondrial Ca2+ handling in Huntington's and Alzheimer's diseases - Role of ER-mitochondria crosstalk
Keywords: هانتینگتن جهش یافته; Mitochondria; Calcium signaling; Endoplasmic reticulum; Mutant huntingtin; Amyloid-beta peptide; Tau;
Modeling Huntington׳s disease with patient-derived neurons
Keywords: هانتینگتن جهش یافته; (HD); Huntington׳s disease; (HTT); Huntingtin; (mtHTT); mutant Huntingtin; (polyQs); polyglutamine repeats; (CNS); central nervous system; (hESC); human embryonic stem cell; (hiPSC); human induced pluripotent stem cell; (polyQs); polyglutamine repeats; (
Systemic study of a natural feedback loop in Huntington's disease at the onset of neurodegeneration
Keywords: هانتینگتن جهش یافته; Grb2; Growth factor receptor bound protein 2; Htt; Huntingtin; mHtt; mutant Huntingtin; FoxD3; Forkhead box D3; Sos-1; son of sevenless homolog 1; Gab1; Grb2 associated binding protein 1; MAPK; mitogen activated protein kinase; ERK; Extra cellular signal
Lycium barbarum polysaccharide attenuates the cytotoxicity of mutant huntingtin and increases the activity of AKT
Keywords: هانتینگتن جهش یافته; Huntington's disease; Mutant huntingtin; AKT; Lycium barbarum polysaccharide
Recombinant Adeno Associated Viral (AAV) vector type 9 delivery of Ex1-Q138-mutant huntingtin in the rat striatum as a short-time model for in vivo studies in drug discovery
Keywords: هانتینگتن جهش یافته; HD; Huntington's disease; mHTT; mutant Huntingtin; rAAV; recombinant Adeno Associated Virus; LV; lentivirus; tNAA; total N-Acetyl Aspartate; Myo-Ins; myoinositol; GFP; green fluorescent protein; NeuN; neuronal nuclei; DARPP-32; dopamine- and cyclic AMP-re
Brain region- and age-dependent dysregulation of p62 and NBR1 in a mouse model of Huntington's disease
Keywords: هانتینگتن جهش یافته; HD; Huntington's disease; htt; huntingtin; LC3; microtubule-associated protein light 3; mhtt; mutant huntingtin; NBR1; Neighbor of BRCA1 Gene 1; polyQ; polyglutamine; Nrf2; nuclear factor (erythroid-derived 2)-like 2; Xpo-1; exportin-1; Aggregates; Autoph
The neurosteroids, allopregnanolone and progesterone, induce autophagy in cultured astrocytes
Keywords: هانتینگتن جهش یافته; Intracellular Zn2+; Mutant huntingtin; Steroids
Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease
Keywords: هانتینگتن جهش یافته; Mutant huntingtin; Abnormal mitochondrial dynamics; Defective axonal transport; RNA silencing; BACHD mouse; Mitochondrial trafficking
P38 MAPK is involved in enhanced NMDA receptor-dependent excitotoxicity in YAC transgenic mouse model of Huntington disease
Keywords: هانتینگتن جهش یافته; aCSF; artificial cerebrospinal fluid; co-IP; co-immunoprecipitation; DIV; days in vitro; ERK; extracellular signal-regulated protein kinase; Ex-NMDARs; extrasynaptic NMDARs; GFP; green fluorescent protein; HD; Huntington disease; HIV-1; human immunodefici
Multiple phenotypes in Huntington disease mouse neural stem cells
Keywords: هانتینگتن جهش یافته; Embryonic stem cell; Mutant huntingtin; Neural stem; Cholesterol; Reactive oxygen species (ROS); Reactive oxygen intermediates (ROI); Motility; Actin; GFAP; Astrocytes
Huntington’s Disease is a disorder of the corpus striatum: Focus on Rhes (Ras homologue enriched in the striatum)
Keywords: هانتینگتن جهش یافته; Huntington Disease; mutant Huntingtin; HIP; HAP; Rhes; mTOR; Striatum; AtrophymHtt, mutant Huntingtin; HIP, Huntingtin interacting proteins; HAP, Huntingtin associated protein; mTOR, mammalian target of Rapamycin; Rhes, Ras homologue enriched in striatum
FK506 ameliorates cell death features in Huntington's disease striatal cell models
Keywords: هانتینگتن جهش یافته; 3-Nitropropionic acid; Cell death; FK506; Mutant huntingtin; Mitochondrial dysfunction; Striatal neurons;
The neurodegenerative process in a neurotoxic rat model and in patients with Huntington's disease: Histopathological parallels and differences
Keywords: هانتینگتن جهش یافته; CAG; cytosine-adenine-guanine; CNP-ase; 2â², 3â²-cyclic nucleotide 3â²-phosphodiesterase; CNS; central nervous system; CN; caudate nucleus; DAB; 3,3â²-diaminobenzidine-tetrahydrochloride; GFAP; glial fibrillary acidic protein; GP; globus pallidus;
ATF3 plays a protective role against toxicity by N-terminal fragment of mutant huntingtin in stable PC12 cell line
Keywords: هانتینگتن جهش یافته; Activating transcription factor 3; Mutant huntingtin; Polyglutamine; Huntington's disease; Cell death
Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
Keywords: هانتینگتن جهش یافته; neurodegeneration; mitochondria; oxidative damage; Huntington's disease; Friedreich ataxia; xeroderma pigmentosum; BDNF; brain-derived neurotrophic factor; CREB; cyclic AMP response element-binding protein; cybrid; cytoplasmic hybrid; FRDA; Friedreich a
Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
Keywords: هانتینگتن جهش یافته; Huntington disease; Neurodegeneration; Mutant huntingtin; Behavior; Neuropathology; Excitotoxicity; Mouse model
Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment
Keywords: هانتینگتن جهش یافته; Striatal degeneration; Lentiviral vector; Mutant huntingtin;