Keywords: پلیگلوتامین; Huntington disease; Huntingtin; Calpains; Calpastatin; Proteolytic cleavage; Aggregation; CAPN; calpain; CAST; calpastatin protein; Cast; mouse calpastatin gene; HD; Huntington disease; HDKI; HdhQ111 knock-in mice; Htt; huntingtin protein; polyQ; polyglut
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Keywords: پلیگلوتامین; Raman; Ultraviolet Raman; Protein; Peptide; Fibrils; Polyglutamine;
Keywords: پلیگلوتامین; 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: پلیگلوتامین; Polyglutamine; β-Hairpin; Oligomers; Primary neurons; Drosophila; Thioflavin T; Amyloid; Huntintin exon 1; Aggregation;
Keywords: پلیگلوتامین; PolyQ; Polyglutamine; Htt; Huntingtin; Httex1; Htt Exon 1; HD; Huntington's disease; PulSA; Pulse Shape Analysis; i; Cells With Inclusions; ni; Cells With Diffuse Httex1; RFU; Relative Fluorescence Units; IPA; Ingenuity Pathway Analysis; FDR; False Discov
Keywords: پلیگلوتامین; AD; Alzheimer's disease; PD; Parkinson's disease; ssNMR; solid-state NMR; α-syn; α-synuclein; β2m; β2-microglobulin; apoA-I; apolipoprotein A-I; polyQ; polyglutamine; IDP; intrinsically disordered protein; htt; huntingtin; MD; molecular dynamics; MHC
Keywords: پلیگلوتامین; ALS; amyotrophic lateral sclerosis; AR; androgen receptor; BDNF; brain-derived neurotrophic factor; CGRP; calcitonin gene-related peptide; ChAT; choline acetyltransferase; DAB; diaminobenzidine; DLN; dorsal nucleus; ENK; enkephalin; GFAP; glial fibrillary
Keywords: پلیگلوتامین; HTT; Huntingtin; MLF; myeloid leukemia factor; HD; Huntington's disease; Poly Q; polyglutamine; GFP; green fluorescent protein; FRAP; fluorescence recovery after photo bleaching; RFI; relative fluorescence intensity; CREB; cAMP response element-binding
Keywords: پلیگلوتامین; Alzheimer disease; Huntington disease; Senescence; Aging; Neurodegeneration; Amyloid; Polyglutamine;
Keywords: پلیگلوتامین; Polyglutamine; Huntington's disease; Disaggregation; Solubilization; Trifluoroacetic acid; Hexafluoroisopropanol; polyGln; polyglutamine; DMSO; dimethyl sulfoxide; TFA; trifluoroacetic acid; HFIP; hexafluoroisopropanol; ThT; thioflavin T; PBS; phosphate-b
Keywords: پلیگلوتامین; HD; Huntington's disease; PolyQ; polyglutamine; FRET; fluorescence resonance energy transfer; Htt; huntingtin protein; CD; circular dichroism; FTIR; Fourier transform infrared spectroscopy; XRD; X-ray diffraction; NMR; nuclear magnetic resonance; AFM; ato
Keywords: پلیگلوتامین; 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: پلیگلوتامین; Spinocerebellar ataxia type 7; Polyglutamine; Haplotype; Founder effect; Africa; Neurodegeneration
Keywords: پلیگلوتامین; AR; androgen receptor; ARE(s); androgen response element(s); bp; basepair; DMS; dimethylsulphate; MMTV; mouse mammary tumor virus; NTD; N-terminal domain; polyQ; polyglutamine; SBMA; spinal and bulbar muscular atrophy; WB; Western blotting; Androgen recep
Keywords: پلیگلوتامین; AD; Alzheimer's disease; ADCA; autosomal dominant cerebellar ataxia; AMPA receptor; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; APP; amyloid precursor protein; Atx2mut; mutant ataxin-2; Aβ peptides; amyloid beta peptides; BACE1; beta
Keywords: پلیگلوتامین; Polyglutamine; Androgen receptor; Spinal and bulbar muscular atrophy; Protein aggregation;
Keywords: پلیگلوتامین; Huntington; Chorea; Huntingtin; Polyglutamine; Striatum; Pathogenesis; Neurodegeneration; Treatment;
Keywords: پلیگلوتامین; misfolding diseases; polyglutamine; protein aggregation; protein-protein interactions;
Keywords: پلیگلوتامین; Polyglutamine; Conformation; Fibril formation; Peptides; IR; CD;
Keywords: پلیگلوتامین; Polyglutamine; Histone; Post-translational modification; KAT; HDAC; DNA methylation; Amelioration
Keywords: پلیگلوتامین; Aβ; β-amyloid; AGE; advanced glycation end product (here: proteins modified by AGEs); Atg; autophagy-related gene; CMA; chaperone-mediated-autophagy; 3-DG; 3-deoxyglucosone; FIP200; focal adhesion kinase family-interacting protein of 200 kDa; IP3; inos
Keywords: پلیگلوتامین; ATN1; atrophin-1; CNS; central nervous system; DRPLA; dentatorubral-pallidoluysian atrophy; HD; Huntington's disease; polyQ; polyglutamine; polyL; polyleucine; SCA; spinocerebellar ataxia; TPEN; N,N,Nâ²,Nâ²-tetrakis (2-pyridylmethyl) ethylenediamine; Z-
Keywords: پلیگلوتامین; Ataxin-3; Molecular dynamics; Polyglutamine; Aggregation; Ubiquitin interacting motif; Intrinsically disordered;
Keywords: پلیگلوتامین; CI; cerebral ischemia; PD; Parkinson's disease; HD; Huntington's disease; SN; substancia nigra; DA; dopamine; GABA; gamma-aminobutyric acid; MSN; medium spiny neuron; CNS; central nervous system; NSC; neural stem cells; ES; embryonic stem; MSC; mesenchyma
Keywords: پلیگلوتامین; Spinal and bulbar muscular atrophy; Androgen receptor; Polyglutamine; CAG repeat; Motoneuron disease; Motoneuron; Neurodegeneration; Protein misfolding; Chaperones; HspB8;
Dual-specificity phosphatase 18 modulates the SUMOylation and aggregation of Ataxin-1
Keywords: پلیگلوتامین; Ataxin-1; Polyglutamine; JNK; Dual-specificity phosphatase 18; SUMO-1;
Intravenous immunoglobulin ameliorates motor and cognitive deficits and neuropathology in R6/2 mouse model of Huntington's disease by decreasing mutant huntingtin protein level and normalizing NF-κB signaling pathway
Keywords: پلیگلوتامین; Huntington's disease; Polyglutamine; Intravenous immunoglobulin; Huntingtin protein;
Polyglutamine repeat proteins disrupt actin structure in Drosophila photoreceptors
Keywords: پلیگلوتامین; Actin; Drosophila; Huntington's disease; Photoreceptor; Formin; Polyglutamine;
A study of Huntington disease-like syndromes in black South African patients reveals a single SCA2 mutation and a unique distribution of normal alleles across five repeat loci
Keywords: پلیگلوتامین; Africa; HD; HDL2; HD phenocopies; Huntington disease; Huntington disease-like 2; Phenocopy; Polyglutamine; Repeat expansions; South Africa;
Pathophysiological consequences of isoform-specific IP3 receptor mutations
Keywords: پلیگلوتامین; ALS; amyotrophic lateral sclerosis; Bcl-2; B-cell lymphoma-2; CA8; carbonic anhydrase 8 (carbonic anhydrase-related protein); CSCC; cervical squamous cell carcinoma; CTT; C-terminal tail; DKO; double knockout; FAD; familial Alzheimer's disease; GS; Gilles
Glutamine addiction activates polyglutamine-based nanocarriers delivering therapeutic siRNAs to orthotopic lung tumor mediated by glutamine transporter SLC1A5
Keywords: پلیگلوتامین; Polyglutamine; Glutamine addiction; Glutamine transporter SLC1A5; Orthotopic lung tumor; RNA inference;
Polyglutamine expansion diseases: More than simple repeats
Keywords: پلیگلوتامین; 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
Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP/p97 in Drosophila melanogaster
Keywords: پلیگلوتامین; 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: پلیگلوتامین; 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
Backbone Engineering within a Latent β-Hairpin Structure to Design Inhibitors of Polyglutamine Amyloid Formation
Keywords: پلیگلوتامین; CP; cross-polarization; MAS; magic angle spinning; polyQ; polyglutamine; ssNMR; solid-state NMR; MAS-NMR; elongation; nucleation; dock-and-lock; trpzip;
Cell-free analysis of polyQ-dependent protein aggregation and its inhibition by chaperone proteins
Keywords: پلیگلوتامین; NAC; nascent polypeptide-associated complex; RAC; ribosome-associated complex; PFD; prefoldin; CCT; chaperonin containing TCP-1; Hsc70; heat shock cognate 71Â kDa protein; Hsc70s; Hsc70 chaperone system; Cell-free; In vitro translation; Polyglutamine; Agg
Conformational modulation mediated by polyglutamine expansion in CAG repeat expansion disease-associated proteins
Keywords: پلیگلوتامین; Huntingtin; Polyglutamine; Conformation; Neurodegeneration; TR-FRET assay; CAG repeat;
Expanded polyglutamine embedded in the endoplasmic reticulum causes membrane distortion and coincides with Bax insertion
Keywords: پلیگلوتامین; Polyglutamine; Membrane; Endoplasmic reticulum; Apoptosis; polyQ; polyglutamine repeats; dox; doxycycline;
Polyglutamine aggregates impair lipid membrane integrity and enhance lipid membrane rigidity
Keywords: پلیگلوتامین; Polyglutamine; Amyloid; Oligomer; Lipid bilayer; Solution AFM; Fluorescence microscopy;
Antioxidant and neuroprotective effects of Dictyophora indusiata polysaccharide in Caenorhabditis elegans
Keywords: پلیگلوتامین; DiPS; Dictyophora indusiata polysaccharide; ROS; reactive oxygen species; MDA; malonaldehyde; SOD; superoxide dismutase; AD; Alzheimer's disease; PD; Parkinson's disease; HD; Huntington's disease; Aβ; amyloid-β protein; polyQ; polyglutamine; Dictyophora
Specific promoter deacetylation of histone H3 is conserved across mouse models of Huntington's disease in the absence of bulk changes
Keywords: پلیگلوتامین; Huntington's disease; Polyglutamine; Epigenetics; Histone acetylation; Histone methylation; Transcriptional dysregulation;
Phenazine derivatives cause proteotoxicity and stress in C. elegans
Keywords: پلیگلوتامین; PD; Parkinson's disease; HD; Huntington's disease; PCA; phenazine-1-carboxylic acid; PCN; pyocyanin; 1-HP; 1-hydroxyphenazine; NAC; N-acetyl cysteine; UPS; ubiquitin proteasome system; UPR; unfolded protein response; Phenazines; C. elegans; ER stress; α-
Prefibrillar huntingtin oligomers isolated from HD brain potently seed amyloid formation
Keywords: پلیگلوتامین; Oligomer; Amyloid; Protein misfolding; Neurodegeneration; Polyglutamine; Huntington's disease;
GAPDH binders as potential drugs for the therapy of polyglutamine diseases: Design of a new screening assay
Keywords: پلیگلوتامین; 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: پلیگلوتامین; 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
Molecular chaperones and neuronal proteostasis
Keywords: پلیگلوتامین; 17-AAG; 17-allylamino-17-demethoxygeldanamycin; 17-DMAG; 17-dimethylamino-ethylamino-17-demethoxygeldanamycin; Aβ; amyloid beta; AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; ARSACS; autosomal recessive spastic ataxia of Charlevoix-Saguena
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: پلیگلوتامین; Ataxin-3; Mouse; Knock-in; Knockin; SCA3; MJD; Ataxia; Spinocerebellar; CAG repeats; Serpina3n; Polyglutamine;
Toward therapeutic targets for SCA3: Insight into the role of Machado–Joseph disease protein ataxin-3 in misfolded proteins clearance
Keywords: پلیگلوتامین; Machado–Joseph disease; Ataxin-3; Polyglutamine; Deubiquitinase; Misfolded protein clearance17-AAG, 17-N-allylamino-17-demethoxygeldanamycin; 17-DMAG, 17-dimethylaminoethylamino-17-demethoxy-geldanamycin; 3-NP, 3-nitropropionic acid; AD, Alzheimer's disea
Autophagy in neurodegenerative diseases: From pathogenic dysfunction to therapeutic modulation
Keywords: پلیگلوتامین; Aβ; amyloid beta; AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; APP; amyloid precursor protein; ATP; adenosine triphosphate; Bci3; Bax cannel inhibitor 3; cAMP; cyclic adenosine monophosphate; CMA; chaperone-mediated autophagy; DLB; dement
The evaluation of polyglutamine repeats in autosomal dominant Parkinson's disease
Keywords: پلیگلوتامین; Trinucleotide repeat diseases; Parkinson's disease; Polyglutamine; Intermediate length; Ataxin-2;