Modeling spinal muscular atrophy in Drosophila links Smn to FGF signaling.
J Cell Biol
; 192(3): 481-95, 2011 Feb 07.
Article
em En
| MEDLINE
| ID: mdl-21300852
ABSTRACT
Spinal muscular atrophy (SMA), a devastating neurodegenerative disorder characterized by motor neuron loss and muscle atrophy, has been linked to mutations in the Survival Motor Neuron (SMN) gene. Based on an SMA model we developed in Drosophila, which displays features that are analogous to the human pathology and vertebrate SMA models, we functionally linked the fibroblast growth factor (FGF) signaling pathway to the Drosophila homologue of SMN, Smn. Here, we characterize this relationship and demonstrate that Smn activity regulates the expression of FGF signaling components and thus FGF signaling. Furthermore, we show that alterations in FGF signaling activity are able to modify the neuromuscular junction defects caused by loss of Smn function and that muscle-specific activation of FGF is sufficient to rescue Smn-associated abnormalities.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Atrofia Muscular Espinal
/
Transdução de Sinais
/
Proteínas de Ligação a RNA
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Proteínas de Drosophila
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Drosophila
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Fatores de Crescimento de Fibroblastos
Tipo de estudo:
Prognostic_studies
Limite:
Animals
Idioma:
En
Revista:
J Cell Biol
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Estados Unidos