Detalhe da pesquisa
1.
Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment.
Brain
; 146(9): 3836-3850, 2023 09 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-36960552
2.
AAV-Mediated CAG-Targeting Selectively Reduces Polyglutamine-Expanded Protein and Attenuates Disease Phenotypes in a Spinocerebellar Ataxia Mouse Model.
Int J Mol Sci
; 25(8)2024 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-38673939
3.
SCA7 Mouse Cerebellar Pathology Reveals Preferential Downregulation of Key Purkinje Cell-Identity Genes and Shared Disease Signature with SCA1 and SCA2.
J Neurosci
; 41(22): 4910-4936, 2021 06 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-33888607
4.
Polyglutamine-expanded ATXN7 alters a specific epigenetic signature underlying photoreceptor identity gene expression in SCA7 mouse retinopathy.
J Biomed Sci
; 29(1): 107, 2022 Dec 20.
Artigo
em Inglês
| MEDLINE | ID: mdl-36539812
5.
Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia.
Mol Ther
; 26(8): 1940-1952, 2018 08 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29853274
6.
Counterregulation between thymic stromal lymphopoietin- and IL-23-driven immune axes shapes skin inflammation in mice with epidermal barrier defects.
J Allergy Clin Immunol
; 138(1): 150-161.e13, 2016 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-26993035
7.
ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo.
Nat Commun
; 8: 15124, 2017 05 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-28492233