Keywords: 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: Spinocerebellar ataxia 2; SCA2; Non-ataxic symptoms; Polyglutamine repeat; CAG, Trinucleotide repeat disorder; AAO; Age at onset; AD; Autosomal dominant; CAG; Cytosine-Adenine-Guanine; CI; Confidence interval; DNA; Deoxyribonucleic acid; ICARS; Internatio
Keywords: 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: 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: 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: 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: 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: 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: AAV4; adeno-associated virus serotype 4; BDNF; brain-derived neurotrophic factor; bFGF; fibroblast growth factor 2; BME; β-mercaptoethanol; BMP; bone morphogenetic protein; Cas9; CRISPR associated protein 9; Cla; claustrum; CN; caudate nucleus; CRISPR; c
Keywords: 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: 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: Autophagy; Aggrephagy; PolyQ; p62; Neurodegeneration; UPS;
Keywords: Ataxin; polyQ; Noncoding; Nucleotide repeat expansion; ALS;
Keywords: Huntington's disease; Human fibroblasts; Gene expression profile; Biomarkers; Huntingtin; Neurodegeneration; PolyQ;
Keywords: 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: 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: 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
Pathophysiological consequences of isoform-specific IP3 receptor mutations
Keywords: 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
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
Backbone Engineering within a Latent β-Hairpin Structure to Design Inhibitors of Polyglutamine Amyloid Formation
Keywords: CP; cross-polarization; MAS; magic angle spinning; polyQ; polyglutamine; ssNMR; solid-state NMR; MAS-NMR; elongation; nucleation; dock-and-lock; trpzip;
A hydrocortisone derivative binds to GAPDH and reduces the toxicity of extracellular polyglutamine-containing aggregates
Keywords: Huntington disease; polyQ; Glyceraldehyde-3-phosphate dehydrogenase; Toxic extracellular aggregates; DARTS; drug affinity responsive target stability; EGFP; enhanced green fluorescent protein; GAPDH; glyceraldehyde-3-phosphate dehydrogenase; GST; glutathi
Expanded polyglutamine embedded in the endoplasmic reticulum causes membrane distortion and coincides with Bax insertion
Keywords: Polyglutamine; Membrane; Endoplasmic reticulum; Apoptosis; polyQ; polyglutamine repeats; dox; doxycycline;
Antioxidant and neuroprotective effects of Dictyophora indusiata polysaccharide in Caenorhabditis elegans
Keywords: 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
GAPDH binders as potential drugs for the therapy of polyglutamine diseases: Design of a new screening assay
Keywords: 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
A plant cell model of polyglutamine aggregation: Identification and characterisation of macromolecular and small-molecule anti-protein aggregation activity in vivo
Keywords: Huntingtin protein; Protein aggregation; PolyQ; LEA protein; sHSP protein; EGCG;
Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3
Keywords: 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: 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
Autophagy in neurodegenerative diseases: From pathogenic dysfunction to therapeutic modulation
Keywords: 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
Inactivation of Apaf1 reduces the formation of mutant huntingtin-dependent aggregates and cell death
Keywords: AkoMEFS; Apaf1 knock-out mice; Apaf1; apoptotic protease-activating factor 1; Cyt c; cytochrome c; DMEM; Dulbecco's Modified Eagle Medium; DOXO; doxorubicin; DTT; dithiothreitol; EDTA; ethylenediaminetetraacetic acid; EGFP; enhanced green fluorescent pr
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
AMPK-α1 functions downstream of oxidative stress to mediate neuronal atrophy in Huntington's disease
Keywords: 3D; three-dimensional; ACC; acety-CoA carboxylase; AD; Alzheimer's disease; AICAR; aminoimidazole carboxamide riboside; AMPK; AMP-activated protein kinase; ALS; amyotrophic lateral sclerosis; ATM; ataxia telangiectasia mutated; CaMKII; Ca2Â +/calmodulin-d
Structural basis for recognition of the third SH3 domain of full-length R85 (R85FL)/ponsin by ataxin-7
Keywords: Atx7; ataxin-7; polyQ; polyglutamine; PRR; proline-rich region; R85FL; full-length R85; SCA7; spinocerebellar ataxia 7; SH3; Src homology 3; Ataxin-7; Proline-rich region; R85FL/ponsin; SH3 domain; Polyglutamine; Inclusion body;
A tale of a tail: Structural insights into the conformational properties of the polyglutamine protein ataxin-3
Keywords: Ataxin-3; PolyQ; Amyloid; Ion mobility spectrometry; Electrospray ionisation-mass spectrometry;
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
Brain region- and age-dependent dysregulation of p62 and NBR1 in a mouse model of Huntington's disease
Keywords: 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
hMTH1 expression protects mitochondria from Huntington's disease-like impairment
Keywords: HD; Huntington disease; Htt; huntingtin; polyQ; polyglutamine; 3-NP; 3-nitropropionic acid; ROS; Reactive oxygen species; 8-oxodG; 8-hydroxyguanine; DDR; DNA damage response; BER; Base excision repair; DSBs; Double strand breaks; DRP1; Dynamin related pro
Levels of supramolecular chirality of polyglutamine aggregates revealed by vibrational circular dichroism
Keywords: PolyQ, polyglutamine; VCD, vibrational circular dichroism; DUVRR, deep UV resonance Raman; IR, infrared; AFM, atomic force microscopy; EM, electron microscopy; H/D, hydrogen-deuteriumPolyglutamine; PolyQ; Fibril aggregate; Chirality; Vibrational circular
Transglutaminase 6 interacts with polyQ proteins and promotes the formation of polyQ aggregates
Keywords: Transglutaminase 6; PolyQ; SCA; Aggregates
Comparative aspects of polyglutamine binding domain in PQBP-1 among Vertebrata
Keywords: polyQ; polyglutamine; PQBP-1; polyglutamine binding protein-1; polyAA; homopolymeric amino acid; PRD; polar amino acid rich domain; NLS; nuclear localization signal; CTD; C-terminal domain; 7aa repeat; seven amino acid repeat; snRNP; small nuclear ribonuc
The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect
Keywords: 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
Computational prediction of the PolyQ and CAG repeat spinocerebellar ataxia network based on sequence identity to untranslated regions
Keywords: SCA; spinocerebellar ataxia; PolyQ; poly glutamine; LTP; long term potentiation; Nt; nucleotide; UTR; untranslated region; DAVID; Database for Annotation, Visualization and Integrated Discovery; CAG; cytosine-adenosine-guanosine; RefSeq; NCBI Referenc
P38 MAPK is involved in enhanced NMDA receptor-dependent excitotoxicity in YAC transgenic mouse model of Huntington disease
Keywords: 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
Toxic effects of expanded ataxin-1 involve mechanical instability of the nuclear membrane
Keywords: SCA1; spinocerebellar ataxia type 1; ATXN1; ataxin 1; polyglutamine; polyQ; SBMA; spinal bulbar muscular atrophy; HD; Huntington disease; DRPLA; dentatorubropallidoluysian atrophy; NLS; nuclear localization signal; RBM17; RNA-binding motif protein 17; AFM
Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins
Keywords: BBB; blood-brain barrier; dAbs; domain antibodies; HD; Huntington's disease; HSC70; heat shock cognate protein 70; HSP70; heat shock protein 70; HTT; huntingtin protein; mHTT; mutant huntingtin protein; PEST; Proline (P), Glutamic Acid (E), Serine (S) a
Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins
Keywords: ALS; amyotrophic lateral sclerosis; AR; androgen receptor; ATG5; autophagy-related gene 5; Bag; Bcl-2 associated athanogene; CBP; CREB-binding protein; CMA; chaperone-mediated autophagy; CMT; Charcot-Marie-Tooth; DAO; d-amino acid oxidase; DCTN1; dynactin
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
Cell-autonomous and non-cell-autonomous toxicity in polyglutamine diseases
Keywords: PolyQ; polyglutamine; SBMA; spinal and bulbar muscular atrophy; SCA; spinocerebellar ataxia; AR; androgen receptor; Hsp; heat shock protein; GnRH; gonadotropin-releasing hormone; LH; luteinizing hormone; FSH; follicle-stimulating hormone; JNK; cJun N-term
Histidine-rich protein Hpn from Helicobacter pylori forms amyloid-like fibrils in vitro and inhibits the proliferation of gastric epithelial AGS cells
Keywords: Aβ; β-amyloid; AD; Alzheimer's disease; ÎΨm; mitochondrial transmembrane potential; FBS; fetal bovine serum; H. pylori; Helicobacter pylori; HRC; histidine-rich Ca2+ binding protein; IAPP; islet amyloid polypeptide; JC-1; 5,5â²,6,6â²-tetrachloro-1,1
A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein
Keywords: ERAD, endoplasmic reticulum-associated degradation; polyQ, expanded polyglutamine proteins; CHX, cycloheximideMITOL; Mitochondrial ubiquitin ligase; PolyQ; Mitochondrial quality-control; ERAD; Apoptosis
Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy
Keywords: AD; alzheimer's disease; AFM; atomic force microscopy; EPCG; (2)-epigallocatechin-3-gallate; GST; glutathione S-transferase; htt; huntingtin; HD; huntington disease; N17; the first 17 amino acids at N-terminus of htt; PD; parkinson's disease; PBS; pho