Expression of human FUS protein in Drosophila leads to progressive neurodegeneration.
Protein Cell
; 2(6): 477-86, 2011 Jun.
Article
em En
| MEDLINE
| ID: mdl-21748598
ABSTRACT
Mutations in the Fused in sarcoma/Translated in liposarcoma gene (FUS/TLS, FUS) have been identified among patients with amyotrophic lateral sclerosis (ALS). FUS protein aggregation is a major pathological hallmark of FUS proteinopathy, a group of neurodegenerative diseases characterized by FUS-immunoreactive inclusion bodies. We prepared transgenic Drosophila expressing either the wild type (Wt) or ALS-mutant human FUS protein (hFUS) using the UAS-Gal4 system. When expressing Wt, R524S or P525L mutant FUS in photoreceptors, mushroom bodies (MBs) or motor neurons (MNs), transgenic flies show age-dependent progressive neural damages, including axonal loss in MB neurons, morphological changes and functional impairment in MNs. The transgenic flies expressing the hFUS gene recapitulate key features of FUS proteinopathy, representing the first stable animal model for this group of devastating diseases.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Envelhecimento
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Células Fotorreceptoras de Invertebrados
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Corpos Pedunculados
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Proteína FUS de Ligação a RNA
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Drosophila melanogaster
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Proteínas Mutantes
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Esclerose Lateral Amiotrófica
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Neurônios Motores
Limite:
Aged
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Animals
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Humans
Idioma:
En
Revista:
Protein Cell
Ano de publicação:
2011
Tipo de documento:
Article