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Isabel Onofre
Isabel Onofre
Sanford Burnham Prebys Medical Discovery Institute
Verified email at sbpdiscovery.org
Title
Cited by
Cited by
Year
Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado–Joseph disease
I Nascimento-Ferreira, T Santos-Ferreira, L Sousa-Ferreira, G Auregan, ...
Brain 134 (5), 1400-1415, 2011
2212011
Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice
C Nóbrega, I Nascimento-Ferreira, I Onofre, D Albuquerque, H Hirai, ...
PloS one 8 (1), e52396, 2013
1372013
Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado–Joseph disease
I Nascimento-Ferreira, C Nobrega, A Vasconcelos-Ferreira, I Onofre, ...
Brain 136 (7), 2173-2188, 2013
1092013
Fibroblasts of Machado Joseph Disease patients reveal autophagy impairment
I Onofre, N Mendonça, S Lopes, R Nobre, JB de Melo, IM Carreira, ...
Scientific reports 6 (1), 28220, 2016
812016
Unravelling endogenous microRNA system dysfunction as a new pathophysiological mechanism in Machado-Joseph disease
V Carmona, J Cunha-Santos, I Onofre, AT Simões, U Vijayakumar, ...
Molecular Therapy 25 (4), 1038-1055, 2017
522017
Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology
C Nóbrega, I Nascimento-Ferreira, I Onofre, D Albuquerque, M Conceição, ...
The Cerebellum 12, 441-455, 2013
452013
RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease
C Nobrega, I Nascimento-Ferreira, I Onofre, D Albuquerque, N Deglon, ...
PLoS One 9 (8), e100086, 2014
422014
Mitochondrial and redox modifications in huntington disease induced pluripotent stem cells rescued by CRISPR/Cas9 CAGs targeting
C Lopes, Y Tang, SI Anjo, B Manadas, I Onofre, LP de Almeida, GQ Daley, ...
Frontiers in cell and developmental biology 8, 576592, 2020
292020
Loss of hierarchical imprinting regulation at the Prader–Willi/Angelman syndrome locus in human iPSCs
D Pólvora-Brandão, M Joaquim, I Godinho, D Aprile, AR Álvaro, I Onofre, ...
Human molecular genetics 27 (23), 3999-4011, 2018
262018
Stem cell-based therapies for polyglutamine diseases
LS Mendonça, I Onofre, CO Miranda, R Perfeito, C Nóbrega, ...
Polyglutamine Disorders, 439-466, 2018
152018
Dissecting the pathogenesis of Machado-Joseph Disease in a new human disease model derived from induced pluripotent stem cells
IMS Onofre
00500:: Universidade de Coimbra, 2016
32016
Protocol for the characterization of the cytosine-adenine-guanine tract and flanking polymorphisms in Machado-Joseph disease: impact on diagnosis and development of gene-based …
SM Lopes, R Faro, MM Lopes, I Onofre, N Mendonça, J Ribeiro, ...
The Journal of Molecular Diagnostics 22 (6), 782-793, 2020
12020
Nuclear aging in polyglutamine-induced neurodegeneration
D Pereira, J Cunha-Santos, A Vasconcelos-Ferreira, J Duarte-Neves, ...
bioRxiv, 2023.06. 09.544056, 2023
2023
RNA Interference Mitigates Motor and Neuropathological Deficits in
C Nóbrega, I Nascimento-Ferreira, I Onofre, D Albuquerque, N Déglon
2014
Silencing of mutant ataxin-3 rescues motor deficits and neurological abnormalities in Machado-Joseph disease transgenic mice
C Nobrega, I Nascimento-Ferreira, I Onofre, D Albuquerque, H Hirai, ...
HUMAN GENE THERAPY 22 (10), A35-A35, 2011
2011
XVIII Annual Congress of the European Society of Gene and Cell Therapy (ESGCT) October 22–25, 2010 Milan, Italy
T Cathomen, T VandenDriessche, A Aartsma-Rus, GJB van Ommen, ...
Human Gene Therapy, 1357-1499, 2010
2010
Activation of autophagy rescues behavioral and neuropathological cerebellar deficits in a lentiviral mouse model of Machado-Joseph disease
I Nascimento-Ferreira, C Nobrega, I Onofre, N Deglon, LP de Almeida
HUMAN GENE THERAPY 21 (10), 1426-1426, 2010
2010
Protocol for the Characterization of the CAG Tract and Flanking Polymorphisms in Machado-Joseph Disease: Impact on Diagnosis and Development of Gene-Based Therapies
SM Lopes, R Faro, MM Lopes, I Onofre, N Mendonça, J Ribeiro, ...
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