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1.
An activator of voltage-gated K+ channels Kv1.1 as a therapeutic candidate for episodic ataxia type 1.
Proc Natl Acad Sci U S A
; 120(31): e2207978120, 2023 08.
Artículo
en Inglés
| MEDLINE | ID: mdl-37487086
2.
Treatment with THI, an inhibitor of sphingosine-1-phosphate lyase, modulates glycosphingolipid metabolism and results therapeutically effective in experimental models of Huntington's disease.
Mol Ther
; 31(1): 282-299, 2023 01 04.
Artículo
en Inglés
| MEDLINE | ID: mdl-36116006
3.
Brain-Periphery Interactions in Huntington's Disease: Mediators and Lifestyle Interventions.
Int J Mol Sci
; 25(9)2024 Apr 25.
Artículo
en Inglés
| MEDLINE | ID: mdl-38731912
4.
Neonatal diagnosis of ACTA2-related disease: A case report and review of literature.
Am J Med Genet A
; 191(4): 1111-1118, 2023 04.
Artículo
en Inglés
| MEDLINE | ID: mdl-36607831
5.
Treatment with the Glycosphingolipid Modulator THI Rescues Myelin Integrity in the Striatum of R6/2 HD Mice.
Int J Mol Sci
; 24(6)2023 Mar 22.
Artículo
en Inglés
| MEDLINE | ID: mdl-36983032
6.
Mutant huntingtin interacts with the sterol regulatory element-binding proteins and impairs their nuclear import.
Hum Mol Genet
; 29(3): 418-431, 2020 02 01.
Artículo
en Inglés
| MEDLINE | ID: mdl-31875875
7.
Glycosphingolipid metabolic reprogramming drives neural differentiation.
EMBO J
; 37(7)2018 04 03.
Artículo
en Inglés
| MEDLINE | ID: mdl-29282205
8.
Polysialic Acid Sustains the Hypoxia-Induced Migration and Undifferentiated State of Human Glioblastoma Cells.
Int J Mol Sci
; 23(17)2022 Aug 24.
Artículo
en Inglés
| MEDLINE | ID: mdl-36076963
9.
Acute manganese treatment restores defective autophagic cargo loading in Huntington's disease cell lines.
Hum Mol Genet
; 28(22): 3825-3841, 2019 11 15.
Artículo
en Inglés
| MEDLINE | ID: mdl-31600787
10.
A Rationale for Hypoxic and Chemical Conditioning in Huntington's Disease.
Int J Mol Sci
; 22(2)2021 Jan 08.
Artículo
en Inglés
| MEDLINE | ID: mdl-33430140
11.
Differential Expression of Sphingolipid Metabolizing Enzymes in Spontaneously Hypertensive Rats: A Possible Substrate for Susceptibility to Brain and Kidney Damage.
Int J Mol Sci
; 22(7)2021 Apr 06.
Artículo
en Inglés
| MEDLINE | ID: mdl-33917593
12.
Inhibition of Ceramide Synthesis Reduces α-Synuclein Proteinopathy in a Cellular Model of Parkinson's Disease.
Int J Mol Sci
; 22(12)2021 Jun 16.
Artículo
en Inglés
| MEDLINE | ID: mdl-34208778
13.
Stimulation of S1PR5 with A-971432, a selective agonist, preserves blood-brain barrier integrity and exerts therapeutic effect in an animal model of Huntington's disease.
Hum Mol Genet
; 27(14): 2490-2501, 2018 07 15.
Artículo
en Inglés
| MEDLINE | ID: mdl-29688337
14.
TUBB3 E410K syndrome: Case report and review of the clinical spectrum of TUBB3 mutations.
Am J Med Genet A
; 182(8): 1977-1984, 2020 08.
Artículo
en Inglés
| MEDLINE | ID: mdl-32573066
15.
Mitochondrial Respiration Changes in R6/2 Huntington's Disease Model Mice during Aging in a Brain Region Specific Manner.
Int J Mol Sci
; 21(15)2020 Jul 30.
Artículo
en Inglés
| MEDLINE | ID: mdl-32751413
16.
Curcumin-Loaded Nanoparticles Based on Amphiphilic Hyaluronan-Conjugate Explored as Targeting Delivery System for Neurodegenerative Disorders.
Int J Mol Sci
; 21(22)2020 Nov 23.
Artículo
en Inglés
| MEDLINE | ID: mdl-33238365
17.
Reduced bioavailable manganese causes striatal urea cycle pathology in Huntington's disease mouse model.
Biochim Biophys Acta Mol Basis Dis
; 1863(6): 1596-1604, 2017 06.
Artículo
en Inglés
| MEDLINE | ID: mdl-28213125
18.
FTY720 (fingolimod) is a neuroprotective and disease-modifying agent in cellular and mouse models of Huntington disease.
Hum Mol Genet
; 23(9): 2251-65, 2014 May 01.
Artículo
en Inglés
| MEDLINE | ID: mdl-24301680
19.
Pridopidine, a dopamine stabilizer, improves motor performance and shows neuroprotective effects in Huntington disease R6/2 mouse model.
J Cell Mol Med
; 19(11): 2540-8, 2015 Nov.
Artículo
en Inglés
| MEDLINE | ID: mdl-26094900
20.
Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice.
Proc Natl Acad Sci U S A
; 109(9): 3528-33, 2012 Feb 28.
Artículo
en Inglés
| MEDLINE | ID: mdl-22331905