Detalhe da pesquisa
1.
Axonal ER Ca2+ Release Selectively Enhances Activity-Independent Glutamate Release in a Huntington Disease Model.
J Neurosci
; 43(20): 3743-3763, 2023 05 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-36944490
2.
Reducing huntingtin by immunotherapy delays disease progression in a mouse model of Huntington disease.
Neurobiol Dis
; 190: 106376, 2024 Jan.
Artigo
em Inglês
| MEDLINE | ID: mdl-38092268
3.
Multicenter expanded access program for access to investigational products for amyotrophic lateral sclerosis.
Muscle Nerve
; 2024 Jun 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-38842106
4.
Allele-specific quantitation of ATXN3 and HTT transcripts in polyQ disease models.
BMC Biol
; 21(1): 17, 2023 02 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-36726088
5.
Mutant Huntingtin Is Cleared from the Brain via Active Mechanisms in Huntington Disease.
J Neurosci
; 41(4): 780-796, 2021 01 27.
Artigo
em Inglês
| MEDLINE | ID: mdl-33310753
6.
Cerebrospinal fluid mutant huntingtin is a biomarker for huntingtin lowering in the striatum of Huntington disease mice.
Neurobiol Dis
; 166: 105652, 2022 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-35143966
7.
Pridopidine rescues BDNF/TrkB trafficking dynamics and synapse homeostasis in a Huntington disease brain-on-a-chip model.
Neurobiol Dis
; 173: 105857, 2022 10 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-36075537
8.
Gene expression profiles complement the analysis of genomic modifiers of the clinical onset of Huntington disease.
Hum Mol Genet
; 29(16): 2788-2802, 2020 09 29.
Artigo
em Inglês
| MEDLINE | ID: mdl-32898862
9.
A Comprehensive Haplotype-Targeting Strategy for Allele-Specific HTT Suppression in Huntington Disease.
Am J Hum Genet
; 105(6): 1112-1125, 2019 12 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-31708117
10.
Length of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease.
Am J Hum Genet
; 104(6): 1116-1126, 2019 06 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-31104771
11.
Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease.
Nucleic Acids Res
; 48(1): 36-54, 2020 01 10.
Artigo
em Inglês
| MEDLINE | ID: mdl-31745548
12.
Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease.
Proc Natl Acad Sci U S A
; 116(19): 9622-9627, 2019 05 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-31015293
13.
Pridopidine reduces mutant huntingtin-induced endoplasmic reticulum stress by modulation of the Sigma-1 receptor.
J Neurochem
; 158(2): 467-481, 2021 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-33871049
14.
Super-resolution imaging reveals extrastriatal synaptic dysfunction in presymptomatic Huntington disease mice.
Neurobiol Dis
; 152: 105293, 2021 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-33556538
15.
Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity.
Neurobiol Dis
; 158: 105479, 2021 10.
Artigo
em Inglês
| MEDLINE | ID: mdl-34390831
16.
Sigma-1 and dopamine D2/D3 receptor occupancy of pridopidine in healthy volunteers and patients with Huntington disease: a [18F] fluspidine and [18F] fallypride PET study.
Eur J Nucl Med Mol Imaging
; 48(4): 1103-1115, 2021 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-32995944
17.
Sigma-1 Receptor (S1R) Interaction with Cholesterol: Mechanisms of S1R Activation and Its Role in Neurodegenerative Diseases.
Int J Mol Sci
; 22(8)2021 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-33920913
18.
Inhibiting cellular uptake of mutant huntingtin using a monoclonal antibody: Implications for the treatment of Huntington's disease.
Neurobiol Dis
; 141: 104943, 2020 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-32407769
19.
HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.
Hum Mol Genet
; 27(2): 239-253, 2018 01 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-29121340
20.
A whole brain longitudinal study in the YAC128 mouse model of Huntington's disease shows distinct trajectories of neurochemical, structural connectivity and volumetric changes.
Hum Mol Genet
; 27(12): 2125-2137, 2018 06 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-29668904