Keywords: HD، بیماری هانتینگتون; RCMκ-CN, reduced and carboxymethylated κ-casein; GS, ginsenosides; Rg1, ginsenoside Rg1; Rb1, ginsenoside Rb1; Re, ginsenoside Re; AD, Alzheimer's disease; PD, Parkinson's disease; HD, Huntington's disease; ThT, thioflavin T; RLS, resonance light scatteri
مقالات ISI HD، بیماری هانتینگتون (ترجمه نشده)
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Keywords: HD، بیماری هانتینگتون; Ataxin-2; Cdk5; SCA2; Phosphorylation; Proteasomal degradation; PolyQALS, amyotrophic lateral sclerosis; Cdk5, cyclin-dependent kinase 5; HD, Huntington's disease; htt, huntingtin; SCA, spinocerebellar ataxia; SDS-PAGE, sodium dodecyl sulfate polyacrylami
Keywords: HD، بیماری هانتینگتون; AD, Alzheimer's disease; ALS, amyotrophic lateral sclerosis; DA, dopaminergic; ER, endoplasmic reticulum; 4E-BP1, eukaryotic initiation factor 4E (eIF4E)-binding protein; HD, Huntington's disease; LRRK2, leucine-rich repeat kinase 2; MPTP, 1-methyl-4-phen
Keywords: HD، بیماری هانتینگتون; PD, Parkinson's disease; HD, Huntington's disease; TS, Tourette's syndrome; SNpc and SNpr, substantia nigra pars compacta and pars reticulata; DA, dopamine; HRT, hormone replacement therapy; MPTP, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine; MA, methamph
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; HTT, huntingtin gene; EDL, extensor digitorum longus muscle; ATP, adenosine triphosphate; ADP, adenosine diphosphate; AMP, adenosine monophosphate; Cr, creatine; PCr, phosphocreatine; PCr/Cr ratio, phosphocreatine/creatine ratio;
Spermidine ameliorates 3-nitropropionic acid (3-NP)-induced striatal toxicity: Possible role of oxidative stress, neuroinflammation, and neurotransmitters
Keywords: HD، بیماری هانتینگتون; 3-NP, 3-Nitropropionic acid; DOPAC, 3,4-dihydroxyphenylacetic acid; 5-HIAA, 5-Hydroxy-3-Indole acetic acid; HVA, Homovanillic Acid; HD, Huntington's disease; IL-6, Interleukin-6; IL-1β, Interleukin-1 < beta >; MSNs, Medium spiny neurons; NMDA, N-methyl-d
Changes in structure and function of diaphragm neuromuscular junctions from BACHD mouse model for Huntington's disease
Keywords: HD، بیماری هانتینگتون; Synaptic vesicles; Neuromuscular junction; Exocytosis; BACHD; Huntington diseaseACh, acetylcholine; htt, huntingtin; mtt, mutant huntingtin; HD, Huntington's disease; MEPP, miniature endplate potential; NMJ, neuromuscular junction; VAChT, vesicular acetyl
Revelation of the IFNα, IL-10, IL-8 and IL-1β as promising biomarkers reflecting immuno-pathological mechanisms in porcine Huntington's disease model
Keywords: HD، بیماری هانتینگتون; AD, assay diluent; CNS, central nervous system; CSF, cerebrospinal fluid; HD, Huntington's disease; HTT, huntingtin protein; mHTT, mutant huntingtin; IKK, inhibitory κB kinase; LPS, lipopolysaccharide; MFI, median fluorescence intensity; NFκB, nuclear fac
Huntingtin interacting protein HYPK is a negative regulator of heat shock response and is downregulated in models of Huntington's Disease
Keywords: HD، بیماری هانتینگتون; AcH4, acetylated histone H4; ChIP, chromatin immunoprecipitation; HD, Huntington's Disease; HTT, Huntingtin; HSE, heat shock element; HSF1, heat shock factor 1; HSPs, heat shock proteins; HSR, heat shock response; HYPK, Huntingtin Yeast Partner K; qRT-PCR
Prostaglandin E2 EP2 activation reduces memory decline in R6/1 mouse model of Huntington's disease by the induction of BDNF-dependent synaptic plasticity
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; PGE2, prostaglandin E2; BDNF, brain-derived neurotrophic factor; htt, huntingtin; mhtt, mutant huntingtin; NORT, novel object recognition test; T-SAT, T-spontaneous alternation task; NIIs, neuronal intra-nuclear inclusions; GFAP,
The neuroprotection of cannabidiol against MPP+-induced toxicity in PC12 cells involves trkA receptors, upregulation of axonal and synaptic proteins, neuritogenesis, and might be relevant to Parkinson's disease
Keywords: HD، بیماری هانتینگتون; AD, Alzheimer's disease; BDNF, brain derived neurotrophic factor; CBD, cannabidiol; FBS, fetal bovine serum; GAP-43, growth associated protein; GDNF, glial-derived neurotrophic factor; HD, Huntington's disease; MPP+, 1-methyl-4-phenylpyridinium iodide; M
Inactivation of adenosine A2A receptors reverses working memory deficits at early stages of Huntington's disease models
Keywords: HD، بیماری هانتینگتون; A2AR, adenosine A2A receptor; aCSF, artificial cerebrospinal fluid; DARPP-32, dopamine- and cAMP-regulated phosphoprotein with molecular weight of 32 kDa; HD, Huntington's disease; KO, knock out; LTD, long-term depression; LTP, long-term potentiation; PN,
Anti-PolyQ Antibodies Recognize a Short PolyQ Stretch in Both Normal and Mutant Huntingtin Exon 1
Keywords: HD، بیماری هانتینگتون; 3D, three-dimensional; DLS, dynamic light scattering; Fab, fragment antigen-binding; HD, Huntington's disease; MALS, multiangle light scattering; polyQ, polyglutamine; SAXS, small-angle X-ray scattering; SEC, size-exclusion chromatography; SSRL, Stanford
A comprehensive glycome profiling of Huntington's disease transgenic mice
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; CDG, congenital disorders of glycosylation; CNS, central nervous system; GSL-omics, glycosphingolipidomics; PolyQ, polyglutamine; TCEP, Tris(2-carboxyethyl) phosphine hydrochloride; MeOH, methanol; ConA, Canavalia ensiformis; LCA
ADIOL protects against 3-NP-induced neurotoxicity in rats: Possible impact of its anti-oxidant, anti-inflammatory and anti-apoptotic actions
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; 3-NP, 3-nitropropionic acid; ADIOL, 5-androstene-3β-17β-diol; DHEA, dehydroepiandrosterone; PPI, prepulse inhibition; iNOS, inducibe nitric oxide synthase; HPF, high power field; H&E, hematoxylin and eosin; DHSB, 3,5-dichloro-2-h
Nucleolar dysfunction in Huntington's disease
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; htt, huntingtin; mthtt, mutant htt; MSNs, medium spiny neurons; rDNA, ribosomal DNA; rRNA, ribosomal RNA; UBF, upstream binding factor; HAT, histone acetyltransferase; Pol I, RNA polymerase I; HMT, histone methyltransferase; CBP,
Nucleotide excision repair and the 26S proteasome function together to promote trinucleotide repeat expansions
Keywords: HD، بیماری هانتینگتون; BER, base excision repair; GG-NER, global genome nucleotide excision repair; HD, Huntington's disease; NER, nucleotide excision repair; R4B, Rad4 binding domain; SC-Trp, synthetic complete medium lacking tryptophan; SCA1, spinocerebellar ataxia type 1; SE
Development and characterization of 3-(benzylsulfonamido)benzamides as potent and selective SIRT2 inhibitors
Keywords: HD، بیماری هانتینگتون; SIRT2; Huntington's disease; 3-(Benzylsulfonamido)benzamides; Polyglutamine aggregation; ADMECNS, central nervous system; HD, Huntington's disease; HDAC, histone deacetylase; SIRT2, sirtuin-2; PolyQ, polyglutamine
The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases
Keywords: HD، بیماری هانتینگتون; AcCoA, Acetyl-coenzyme A; AD, Alzheimer's disease; ALS, Amyotrophic lateral sclerosis; ATP, Adenosine triphosphate; DA, Dopamine; ETC, Electron transport chain; FADH, Flavin adenine dinucleotide; FRDA, Freidreich's ataxia; HD, Huntington's disease; IMS, I
Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death
Keywords: HD، بیماری هانتینگتون; HTT, Huntingtin; HD, Huntington's disease; ES, Embryonic stem; NS, Neural stem; iPS, Induced pluripotent stem cell; BLBP, Brain lipid-binding protein; SOX2, SRY (sex determining region Y)-box 2; GFAP, Glial fibrillary acidic protein; BDNF, Brain-derived n
Scientific and ethical issues related to stem cell research and interventions in neurodegenerative disorders of the brain
Keywords: HD، بیماری هانتینگتون; hES, human embryonic stem cells; HD, Huntington's disease; iPS, induced pluripotent stem cells; iN, induced neurons; PD, Parkinson's disease; VM, Ventral mesencephalonStem cells; Parkinson's disease; Neural grafting; Disease modelling; Clinical trials; Et
Impaired basal and running-induced hippocampal neurogenesis coincides with reduced Akt signaling in adult R6/1 HD mice
Keywords: HD، بیماری هانتینگتون; AHN, adult hippocampal neurogenesis; BrdU, 5-bromodeoxyuridine; BS, blocking solution; DCX, doublecortin; DG, dentate gyrus; GFAP, glial fibrillary acid protein; GCL, granule cell layer; GH, growth hormone; HD, Huntington's disease; IGF-1, insulin-like gr
Longitudinal assessment of gait abnormalities following penetrating ballistic-like brain injury in rats
Keywords: HD، بیماری هانتینگتون; TBI, traumatic brain injury; PBBI, penetrating ballistic-like brain injury; PD, Parkinson's disease; HD, Huntington's disease; CST, corticospinal tract; SCI, spinal cord injury; CCI, controlled cortical impact; NRC, National Research Council; NS, neurolog
Electroencephalography of response inhibition tasks: Functional networks and cognitive contributions
Keywords: HD، بیماری هانتینگتون; aMCC, anterior midcingulate cortex; BA, Brodmann area; CPT, continuous performance test; EEG, electroencephalography; ERP, event-related potential; fMRI, functional magnetic resonance imaging; HD, Huntington's disease; IFC, inferior frontal cortex; MCC, m
Axonal transport and neurodegenerative disease: Can we see the elephant?
Keywords: HD، بیماری هانتینگتون; HD, Huntington's Disease; AD, Alzheimer's Disease; APP, amyloid precursor protein; TGN, trans-golgi network; BACE, beta-secretaseAlzheimer's disease; Huntington's disease; Axonal transport; Kinesin; Dynein
CCG polymorphisms in the huntingtin gene have no effect on the pathogenesis of patients with Huntington's disease in mainland Chinese families
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; polyQ, polyglutamine; htt, huntingtin(protein); HTT, huntingtin(gene); HEK293T, human embryonic kidney 293T cells; PCR, polymerase chain reaction; GFP, green fluorescent protein; DMEM, Dulbecco's modified Eagle's medium; FBS, fet
Epigenetic histone acetylation and deacetylation mechanisms in experimental models of neurodegenerative disorders
Keywords: HD، بیماری هانتینگتون; MPP+, 1-methyl-4-phenylpyridinium; 4-PBA, 4-phenylbutyrate; AD, Alzheimer's disease; Aβ, amyloid-beta peptide; APP, β-amyloid precursor protein; HAT, histone acetyl transferase; HDACs, histone deacetylases; HD, Huntington's disease; PD, Parkinson's diseas
Brain Derived Neurotrophic Factor: A novel neurotrophin involved in psychiatric and neurological disorders
Keywords: HD، بیماری هانتینگتون; AD, Alzheimer's Disease; BDNF, Brain Derived Neurotrophic Factor; BDNFMet, Valine to Methionine substitution at position 66 in the pro-domain of BDNF gene; CSF, Cerebrospinal fluid; DT, Delirium Tremens; HD, Huntington's Disease; mBDNF, Proteolytically pr
An open system for automatic home-cage behavioral analysis and its application to male and female mouse models of Huntington's disease
Keywords: HD، بیماری هانتینگتون; AD, Alzheimer disease; PD, Parkinson's disease; HD, Huntington's disease; WT, wild-typeMouse behavior; Home cage behavior; Video-based automated behavior phenotyping; Huntington's disease; R6/2 transgenic mouse
Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA)
Keywords: HD، بیماری هانتینگتون; SBMA, spinal and bulbar muscular atrophy; AR, androgen receptor; HD, Huntington's disease; AF, transcativation function; CBP, c-AMP response element binding protein-binding protein; NI, nuclear inclusion; N/C, amino- and carboxyl-terminal domains; Hsp, he
Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway
Keywords: HD، بیماری هانتینگتون; iPS, induced pluripotent stem cells; HD, Huntington's disease; wt, wild type; HTT, huntingtin; BDNF, brain-derived neurotrophic factor; PH3, phospho-histone H3; PI, propidium iodide; GFAP, glial fibrillary acid protein; MAP2ab, microtubule-associated prot
The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington's disease patients demonstrates mutation related enhanced lysosomal activity
Keywords: HD، بیماری هانتینگتون; iPS, induced pluripotent stem; HD, Huntington's disease; WT, wild type; HTT, huntingtin; PH3, phospho-histone H3; MEF, mouse embryonic fibroblasts; EBs, embryoid bodies; ES, embryonic stemHuman induced pluripotent stem cells; Huntington's disease; Neurona
Tissue transglutaminase overexpression does not modify the disease phenotype of the R6/2 mouse model of Huntington's disease
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; htt, huntingtin; polyQ, polyglutamine; TG2, tissue transglutaminase; WT, wild type; MSN, medium spiny neuronHuntington's disease; Transglutaminase; Mouse model; Phenotype; Modifiers; Aggregates
Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; htt, huntingtin; mhtt, mutant huntingtin; htt552, N-terminal huntingtin fragment 1–552 aa; BDNF, brain-derived neurotrophic factor; ACM, astrocyte conditioned mediumAstrocytes; Huntington's disease; Brain-derived neurotrophic fac
Metal ions as modulators of protein conformation and misfolding in neurodegeneration
Keywords: HD، بیماری هانتینگتون; Aβ, amyloid-β peptide; AD, Alzheimer's disease; ALS, amyotrophic lateral sclerosis; APP, amyloid precursor protein; CJD, Creutzfeldt–Jakob disease; CNS, central nervous system; CSF, cerebrospinal fluid; HD, Huntington's disease; IDP, intrinsically disorde
Physical Chemistry of Polyglutamine: Intriguing Tales of a Monotonous Sequence
Keywords: HD، بیماری هانتینگتون; polyQ, polyglutamine; HD, Huntington's disease; htt, huntingtin; FCS, fluorescence correlation spectroscopy; httNT, htt 17-amino acid N-terminus; AUC, analytical ultracentrifugation; GFP, green fluorescent protein; FTIR, Fourier transform infrared spectro
IGF-1 protects against diabetic features in an in vivo model of Huntington's disease
Keywords: HD، بیماری هانتینگتون; BACHD, bacterial artificial chromosome Huntington's disease; BDNF, brain-derived neurotrophic factor; CAG, cytosine–adenine–guanine trinucleotide; CNS, central nervous system; GH, growth hormone; HD, Huntington's disease; HPA, hypothalamic–pituitary–adren
Amyloid in neurodegenerative diseases: Friend or foe?
Keywords: HD، بیماری هانتینگتون; htt, huntingtin; HD, Huntington's disease; AD, Alzheimer's disease; CC, coiled-coil; (polyQ), polyglutamine; IB, inclusion body; APP, amyloid precursor protein; FRET, fluorescence resonance energy transfer; LTP, long-term potentiation; PQC, protein qualit
Neurogenesis in the R6/2 mouse model of Huntington's disease is impaired at the level of NeuroD1
Keywords: HD، بیماری هانتینگتون; huntingtin; hippocampus; neuroblasts; transcriptional dysregulation; transgenic miceBmal1, brain and muscle Arnt-like protein-1; Dcx, doublecortin; DG, dentate gyrus; HAP1, huntingtin associated protein 1; HD, Huntington's disease; Htt, huntingtin; mPer1,
Gene–environment interactions and construct validity in preclinical models of psychiatric disorders
Keywords: HD، بیماری هانتینگتون; COMT, catechol-O-methyltransferase; THC, Delta-9-tetrahydrocannabinol; HD, Huntington's disease; SNPs, single nucleotide polymorphisms; GWA, genome-wide association; TRPs, tandem repeat polymorphisms; SSRIs, selective serotonin reuptake inhibitorsAnimal m
Lentiviral delivery of Meteorin protects striatal neurons against excitotoxicity and reverses motor deficits in the quinolinic acid rat model
Keywords: HD، بیماری هانتینگتون; CDNF, cerebral dopamine neurotrophic factor; CNTF, ciliary neurotrophic factor; GDNF, Glial Cell Line-derived Neurotrophic Factor; GFP, green fluorescent protein; HD, Huntington's Disease; MANF, mesencephalic astrocyte-derived neurotrophic factor; PD, Par
The Rate of PolyQ-Mediated Aggregation Is Dramatically Affected by the Number and Location of Surrounding Domains
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; SCA, spinocerebellar ataxia; NCBI NR, National Center for Biotechnology Information's Non-Redundant protein database; SpA, Staphylococcus aureus Protein Apolyglutamine; protein aggregation; fibrils; conformational disease; misfol
A New Apo-Caspase-6 Crystal Form Reveals the Active Conformation of the Apoenzyme
Keywords: HD، بیماری هانتینگتون; HD, Huntington's disease; mHTT, mutant huntingtin; PDB, Protein Data Bankcaspases; Huntington's disease; Alzheimer's disease; caspase-6; crystal structure
Protein aggregate spreading in neurodegenerative diseases: Problems and perspectives
Keywords: HD، بیماری هانتینگتون; CJD, Creutzfeldt–Jakob disease; AD, Alzheimer's disease; PD, Parkinson's disease; HD, Huntington's disease; Aβ, amyloid-β; polyQ, polyglutamine; CNS, central nervous system; NFTs, neurofibrillary tangles; NTs, neuropil threads; APP, amyloid precursor prot
Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease
Keywords: HD، بیماری هانتینگتون; ANOVA, analysis of variance; ANS, autonomic nervous system; CT, circadian time; DD, constant darkness; ECG, electrocardiogram; HD, Huntington's disease; HR, heart rate; HRV, heart rate variability; IHC, immunohistochemistry; LD, light–dark; PER2, PERIOD2;
No change in progenitor cell proliferation in the hippocampus in Huntington's disease
Keywords: HD، بیماری هانتینگتون; subgranular zone; hippocampus; Huntington's disease; subventricular zone; mood disorder; stem cellBcl-2, B-cell lymphoma 2; DAB, 3,3 diaminobenzidine chromogen; DG, dentate gyrus; GCL, granule cell layer; HD, Huntington's disease; MCM2, mini-chromosome ma
Multivesicular bodies in neurons: Distribution, protein content, and trafficking functions
Keywords: HD، بیماری هانتینگتون; Aβ42, amyloid–beta 42 peptide; AD, Alzheimer's disease; CNS, central nervous system; ESCRT, endosomal sorting complex required for transport; FA, fractional area; HD, Huntington's disease; HRP, horseradish peroxidase; LBPA, lysobisphosphatidic acid; MVB,
Systemic administration of 3-nitropropionic acid points out a different role for active caspase-3 in neurons and astrocytes
Keywords: HD، بیماری هانتینگتون; 3-NPA, 3-Nitropropionic acid; AD, Alzheimer's disease; GFAP, Glial fibrillary acidic protein; HD, Huntington's disease; MCC, Mitochondrial respiratory chain complex; NeuN, Neuronal nuclei protein; PBS, Phosphate buffered saline; PCD, Programmed cell death
Brain region specificity of 3-nitropropionic acid-induced vulnerability of neurons involves cytochrome c oxidase
Keywords: HD، بیماری هانتینگتون; 18S rRNA, 18S ribosomal RNA; cDNA, first strand complementary DNA; COX, cytochrome c oxidase; DEPC, diethyl pyrocarbonate; FCS, fetal calf serum; H2DCHF, dihydrodichlorofluorescein diacetate; HD, Huntington's disease; Hoechst, Hoechst 33342 trihydrochlori
Cystamine prevents MPTP-induced toxicity in young adult mice via the up-regulation of the brain-derived neurotrophic factor
Keywords: HD، بیماری هانتینگتون; BDNF, brain-derived neurotrophic factor; cRNA, complementary RNA; DAB, 3,3′ diaminobenzidine tetrahydrochloride; DAergic, dopaminergic; HD, Huntington's disease; HSJ1b, heat shock DnaJ-containing protein 1b; Hsp, heat shock protein; MPTP, 1-methyl-4-pheny