| Nome |
# |
|
The coding and long noncoding single-cell atlas of the developing human fetal striatum, file dfa8b9a8-ec11-748b-e053-3a05fe0a3a96
|
867
|
|
ADAM10 hyperactivation acts on piccolo to deplete synaptic vesicle stores in Huntington's disease, file dfa8b9a6-0361-748b-e053-3a05fe0a3a96
|
504
|
|
Phylogenetic comparison of huntingtin homologues reveals the appearance of a primitive polyQ in sea urchin, file dfa8b990-7144-748b-e053-3a05fe0a3a96
|
450
|
|
Evaluation of histone deacetylases as drug targets in Huntington's disease models : Study of HDACs in brain tissues from R6/2 and CAG140 knock-in HD mouse models and human patients and in a neuronal HD cell model, file dfa8b990-6493-748b-e053-3a05fe0a3a96
|
435
|
|
Developmentally coordinated extrinsic signals drive human pluripotent stem cell differentiation toward authentic DARPP-32(+) medium-sized spiny neurons, file dfa8b990-98d2-748b-e053-3a05fe0a3a96
|
426
|
|
Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease, file dfa8b98e-817d-748b-e053-3a05fe0a3a96
|
412
|
|
EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs, file dfa8b991-21ec-748b-e053-3a05fe0a3a96
|
360
|
|
Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease, file dfa8b990-165f-748b-e053-3a05fe0a3a96
|
313
|
|
Loss of huntingtin function complemented by small molecules acting as repressor element 1/neuron restrictive silencer element silencer modulators, file dfa8b98e-817b-748b-e053-3a05fe0a3a96
|
308
|
|
Neural stem and progenitor cells: choosing the right Shc, file dfa8b990-5030-748b-e053-3a05fe0a3a96
|
298
|
|
Niche-independent symmetrical self-renewal of a mammalian tissue stem cell, file dfa8b98f-66f3-748b-e053-3a05fe0a3a96
|
273
|
|
Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis, file dfa8b990-86dd-748b-e053-3a05fe0a3a96
|
252
|
|
Wild-type huntingtin protects from apoptosis upstream of caspase-3, file dfa8b990-574f-748b-e053-3a05fe0a3a96
|
248
|
|
Pitfalls in the detection of cholesterol in Huntington’s disease models, file dfa8b98f-57cb-748b-e053-3a05fe0a3a96
|
236
|
|
REST Controls Self-Renewal and Tumorigenic Competence of Human Glioblastoma Cells, file dfa8b990-5742-748b-e053-3a05fe0a3a96
|
231
|
|
Activation of the JAK/STAT pathway leads to proliferation of ST14A central nervous system progenitor cells, file dfa8b990-4fbf-748b-e053-3a05fe0a3a96
|
223
|
|
Huntingtin’s neuroprotective activity occurs via inhibition of pro-caspase 9 processing, file dfa8b990-6ae1-748b-e053-3a05fe0a3a96
|
219
|
|
Adaptation of NS cells growth and differentiation to high-throughput screening-compatible plates, file dfa8b98f-1700-748b-e053-3a05fe0a3a96
|
207
|
|
Characterization, developmental expression and evolutionary features of the huntingtin gene in the amphioxus Branchiostoma floridae, file dfa8b98f-7927-748b-e053-3a05fe0a3a96
|
205
|
|
Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes, file dfa8b99a-cd8a-748b-e053-3a05fe0a3a96
|
199
|
|
Cholesterol-loaded nanoparticles ameliorate synaptic and cognitive function in Huntington's disease mice, file dfa8b997-316a-748b-e053-3a05fe0a3a96
|
175
|
|
Expression and activation of SH2/PTB-containing ShcA adaptor protein reflects the pattern of neurogenesis in the mammalian brain, file dfa8b990-574c-748b-e053-3a05fe0a3a96
|
173
|
|
Human cortical organoids expose a differential function of GSK3 on cortical neurogenesis, file dfa8b9a0-7ff8-748b-e053-3a05fe0a3a96
|
173
|
|
Expressed Alu repeats as a novel, reliable tool for normalization of real-time quantitative RT-PCR data, file dfa8b98f-16f9-748b-e053-3a05fe0a3a96
|
166
|
|
Proteasome activator enhances survival of huntington's disease neuronal model cells, file dfa8b98e-816d-748b-e053-3a05fe0a3a96
|
155
|
|
ErbB4 expression in neural progenitor cells (ST14A) is necessary to mediate neuregulin-1beta1-induced migration, file dfa8b996-1608-748b-e053-3a05fe0a3a96
|
154
|
|
A CRISPR-strategy for the generation of a detectable fluorescent hESC reporter line (WAe009-A-37) for the subpallial determinant GSX2, file dfa8b9a5-1559-748b-e053-3a05fe0a3a96
|
154
|
|
Huntingtin gene evolution in Chordata and its peculiar features in the ascidian Ciona genus, file dfa8b990-7146-748b-e053-3a05fe0a3a96
|
144
|
|
Brain-derived neurotrophic factor in patients with Huntington's disease, file dfa8b993-a64f-748b-e053-3a05fe0a3a96
|
144
|
|
Phosphorylation of huntingtin at residue T3 is decreased in Huntington’s disease and modulates mutant huntingtin protein conformation, file dfa8b99d-d2c5-748b-e053-3a05fe0a3a96
|
129
|
|
Inhibiting pathologically active ADAM10 rescues synaptic and cognitive decline in Huntington’s disease, file dfa8b99e-ddd1-748b-e053-3a05fe0a3a96
|
128
|
|
Pathogenesis, file dfa8b998-175d-748b-e053-3a05fe0a3a96
|
126
|
|
Genome-wide definition of promoter and enhancer usage during neural induction of human embryonic stem cells, file dfa8b99e-084c-748b-e053-3a05fe0a3a96
|
123
|
|
Efficacy of Cholesterol Nose-to-Brain Delivery for Brain Targeting in Huntington's Disease, file dfa8b9a0-ea3d-748b-e053-3a05fe0a3a96
|
123
|
|
null, file dfa8b991-1fc4-748b-e053-3a05fe0a3a96
|
122
|
|
Inhibiting pathologically active ADAM10 rescues synaptic and cognitive decline in Huntington’s disease, file dfa8b99e-8bf0-748b-e053-3a05fe0a3a96
|
117
|
|
New label-free methods for protein relative quantification applied to the investigation of an animal model of Huntington Disease, file dfa8b9a3-24de-748b-e053-3a05fe0a3a96
|
117
|
|
Brain Regional Identity and Cell Type Specificity Landscape of Human Cortical Organoid Models, file 5a39f1a7-2e29-4feb-aba9-7ce6dab1a268
|
107
|
|
hiPSCs for predictive modelling of neurodegenerative diseases : dreaming the possible, file dfa8b9a8-600b-748b-e053-3a05fe0a3a96
|
106
|
|
Striatal infusion of cholesterol promotes dose-dependent behavioral benefits and exerts disease-modifying effects in Huntington's disease mice, file dfa8b9a3-bc12-748b-e053-3a05fe0a3a96
|
102
|
|
RUES2 hESCs exhibit MGE-biased neuronal differentiation and muHTT-dependent defective specification hinting at SP1, file dfa8b9a5-1d18-748b-e053-3a05fe0a3a96
|
94
|
|
Stem Cell-Derived Human Striatal Progenitors Innervate Striatal Targets and Alleviate Sensorimotor Deficit in a Rat Model of Huntington Disease, file dfa8b9a4-c28c-748b-e053-3a05fe0a3a96
|
81
|
|
Insights into kinetics, release, and behavioral effects of brain-targeted hybrid nanoparticles for cholesterol delivery in Huntington disease, file dfa8b9a4-9630-748b-e053-3a05fe0a3a96
|
80
|
|
iPSC-derived neural precursors exert a neuroprotective role in immune-mediated demyelination via the secretion of LIF, file dfa8b9a1-5058-748b-e053-3a05fe0a3a96
|
76
|
|
The evolutionary history of the polyQ tract in huntingtin sheds light on its functional pro-neural activities, file dfa8b9aa-4264-748b-e053-3a05fe0a3a96
|
75
|
|
SREBP2 delivery to striatal astrocytes normalizes transcription of cholesterol biosynthesis genes and ameliorates pathological features in Huntington’s Disease, file dfa8b9a4-9054-748b-e053-3a05fe0a3a96
|
70
|
|
A transgenic minipig models of Huntington’s disease, file dfa8b991-2332-748b-e053-3a05fe0a3a96
|
54
|
|
Nutritional assessment in idiopathic pulmonary fibrosis: a prospective multicentre study, file dfa8b9aa-2498-748b-e053-3a05fe0a3a96
|
53
|
|
Neural stem cells engrafted in the adult brain fuse with endogenous neurons, file dfa8b991-25fe-748b-e053-3a05fe0a3a96
|
48
|
|
Chronic cholesterol administration to the brain supports complete and long-lasting cognitive and motor amelioration in Huntington's disease, file 5034147e-7575-4519-a36d-8d5269f134d7
|
38
|
|
Successful sequencing of Huntington’s disease CAG microsatellite orthologs from museum collection specimens, file dfa8b997-5435-748b-e053-3a05fe0a3a96
|
31
|
|
Cholesterol-laden brain-permeable nanoparticles support long-lasting cognitive recovery and motor amelioration in the slow-progressing zQ175DN mouse model of Huntington’s Disease, file 3742d857-55ac-4204-b91a-70b326b86214
|
27
|
|
Modeling human telencephalic development and autism-associated SHANK3 deficiency using organoids generated from single neural rosettes, file b97d8bea-5daf-4bc1-9ef1-1a23276f387b
|
23
|
|
Dose-dependent and disease-modifying effects of striatal infusion of cholesterol in Huntington{\textquotesingle}s disease, file 4889e0a1-744f-49d8-953c-5f2d21220de2
|
21
|
|
In vitro-derived medium spiny neurons recapitulate human striatal development and complexity at single-cell resolution, file 3b2e1bef-b5ff-4f8e-ad04-e6e6854b6f50
|
19
|
|
Induced pluripotent stem cells from patients with Huntington’s disease: show CAG repeat-expansion-associated phenotypes, file dfa8b993-8743-748b-e053-3a05fe0a3a96
|
19
|
|
Expression of the JAK and STAT superfamilies in human meningiomas, file dfa8b990-501e-748b-e053-3a05fe0a3a96
|
8
|
|
In vitro and in vivo models of Huntington’s disease show alterations in the endocannabinoid system, file dfa8b991-191a-748b-e053-3a05fe0a3a96
|
8
|
|
Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease : a cross-validation study in Q175 knock-in mice, file dfa8b997-8d66-748b-e053-3a05fe0a3a96
|
8
|
|
Signalling through the JAK-STAT pathway in the developing brain, file dfa8b990-5466-748b-e053-3a05fe0a3a96
|
7
|
|
Human pluripotent stem cell differentiation into authentic striatal projection neurons, file dfa8b991-2604-748b-e053-3a05fe0a3a96
|
7
|
|
Binding of the repressor complex REST-mSIN3b by small molecules restores neuronal gene transcription in Huntington's disease models, file dfa8b993-826f-748b-e053-3a05fe0a3a96
|
7
|
|
Forkhead transcription factor FOXO3a levels are increased in Huntington disease because of overactivated positive autofeedback loop, file dfa8b993-d8f3-748b-e053-3a05fe0a3a96
|
7
|
|
Foreword, file dfa8b98f-c288-748b-e053-3a05fe0a3a96
|
6
|
|
Members of the JAK/STAT proteins are expressed and regulated during development in the mammalian forebrain, file dfa8b990-502e-748b-e053-3a05fe0a3a96
|
6
|
|
Allele-specific silencing as treatment for gene duplication disorders : Proof-of-principle in autosomal dominant leukodystrophy, file dfa8b9a1-5c25-748b-e053-3a05fe0a3a96
|
6
|
|
Shc signaling in differentiating neural progenitor cells, file dfa8b990-508b-748b-e053-3a05fe0a3a96
|
5
|
|
Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors, file dfa8b993-898d-748b-e053-3a05fe0a3a96
|
5
|
|
hESC-derived striatal progenitors grafted into a Huntington’s disease rat model support long-term functional motor recovery by differentiating, self-organizing and connecting into the lesioned striatum, file 3391490a-600b-4ded-b4d3-7a5317029de4
|
4
|
|
Ciliary neurotrophic factor may activate mature astrocytes via binding with the leukemia inhibitory factor receptor, file dfa8b990-4fbb-748b-e053-3a05fe0a3a96
|
4
|
|
hESC-derived striatal progenitors grafted into a Huntington’s disease rat model support long-term functional motor recovery by differentiating, self-organizing and connecting into the lesioned striatum, file 1acbac97-94a9-424d-bba0-41a91fda6015
|
3
|
|
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes, file dfa8b990-5023-748b-e053-3a05fe0a3a96
|
3
|
|
NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease, file dfa8b993-700f-748b-e053-3a05fe0a3a96
|
3
|
|
Repressor element-1 silencing transcription factor (REST) is present in human control and Huntington’s
disease neurones, file dfa8b993-8994-748b-e053-3a05fe0a3a96
|
3
|
|
An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10-Ncadherin, file dfa8b993-b2c0-748b-e053-3a05fe0a3a96
|
3
|
|
Sex-specific effects of the Huntington gene on normal neurodevelopment, file dfa8b99d-ee45-748b-e053-3a05fe0a3a96
|
3
|
|
DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis, file dfa8b9a4-b035-748b-e053-3a05fe0a3a96
|
3
|
|
A cutting-edge approach based on UHPLC-MS to simultaneously investigate oxysterols and cholesterol precursors in biological samples: Validation in Huntington's disease mouse model, file 1dbf7fb9-e3a7-4b00-8899-c5ac91d449b2
|
2
|
|
null, file dfa8b990-53f9-748b-e053-3a05fe0a3a96
|
2
|
|
In vivo delivery of DN:REST improves transcriptional changes of REST-regulated genes in HD mice, file dfa8b993-697c-748b-e053-3a05fe0a3a96
|
2
|
|
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, file dfa8b993-6cb2-748b-e053-3a05fe0a3a96
|
2
|
|
Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death, file dfa8b993-6ffb-748b-e053-3a05fe0a3a96
|
2
|
|
Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease, file dfa8b993-8551-748b-e053-3a05fe0a3a96
|
2
|
|
Dynamic and Cell-Specific DACH1 Expression in Human Neocortical and Striatal Development, file dfa8b99e-05e9-748b-e053-3a05fe0a3a96
|
2
|
|
Huntingtin gene CAG repeat size affects autism risk: Family-based and case–control association study, file dfa8b9a3-45d4-748b-e053-3a05fe0a3a96
|
2
|
|
null, file dfa8b990-5087-748b-e053-3a05fe0a3a96
|
1
|
|
null, file dfa8b990-5089-748b-e053-3a05fe0a3a96
|
1
|
|
null, file dfa8b990-508e-748b-e053-3a05fe0a3a96
|
1
|
|
null, file dfa8b990-574e-748b-e053-3a05fe0a3a96
|
1
|
|
Whole body cholesterol metabolism is impaired in Huntington's disease, file dfa8b993-6ead-748b-e053-3a05fe0a3a96
|
1
|
|
Emerging roles for cholesterol in Huntington's disease, file dfa8b993-7b30-748b-e053-3a05fe0a3a96
|
1
|
|
Stamina therapies: Let the record stand, file dfa8b993-8b68-748b-e053-3a05fe0a3a96
|
1
|
|
High throughput screening for inhibitors of REST in neural derivatives of human embryonic stem cells reveals a chemical compound that promotes expression of neuronal genes, file dfa8b993-95e3-748b-e053-3a05fe0a3a96
|
1
|
|
Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway, file dfa8b993-95e4-748b-e053-3a05fe0a3a96
|
1
|
|
Wnt5a is a transcriptional target of Dlx homeogenes and promotes differentiation of interneuron progenitors in vitro and in vivo, file dfa8b993-c8e9-748b-e053-3a05fe0a3a96
|
1
|
|
Peroxisome-proliferator-activated receptor gamma coactivator 1 α contributes to dysmyelination in experimental models of Huntington's disease, file dfa8b993-d57a-748b-e053-3a05fe0a3a96
|
1
|
|
Huntingtin gene and its CAG repeats number in Primates, file dfa8b997-17c7-748b-e053-3a05fe0a3a96
|
1
|
|
Normal Function of Huntingtin, file dfa8b997-ecde-748b-e053-3a05fe0a3a96
|
1
|
|
L16 Identifying a therapeutic regimen for cholesterol delivery to Huntington’s disease brain, file dfa8b998-34eb-748b-e053-3a05fe0a3a96
|
1
|
|
HTT evolution and brain development, file dfa8b998-4d87-748b-e053-3a05fe0a3a96
|
1
|
| Totale |
10.353 |