Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Añadir filtros








Intervalo de año
1.
Experimental & Molecular Medicine ; : 348-356, 2006.
Artículo en Inglés | WPRIM | ID: wpr-53156

RESUMEN

Gaucher disease is a glycosphingolipid storage disease caused by deficiency of glucocerebrosidase, resulting in the accumulation of glucosylceramide in lysosomes. The neuronopathic forms of this disease are associated with neuronal loss and neurodegeneration. However, the pathophysiological mechanisms leading to prenatal and neonatal death remain uncharacterized. To investigate brain dysfunction in Gaucher disease, we studied the effects of neurotrophic factors during development in a mouse model of Gaucher disease. The expression of brain-derived neurotrophic factor and nerve growth factor was reduced in the cerebral cortex, brainstem, and cerebellum of Gaucher mice, compared with that in wild-type mice. Extracellular signal-regulated kinase (ERK) 1/2 expression was downregulated in neurons from Gaucher mice and correlated with a decreased number of neurons. These results suggest that a reduction in neurotrophic factors could be involved in neuronal loss in Gaucher disease.


Asunto(s)
Ratones , Animales , Transducción de Señal , Neuronas/metabolismo , Factores de Crecimiento Nervioso/metabolismo , Modelos Animales , Ratones Endogámicos C57BL , Sistema de Señalización de MAP Quinasas/fisiología , Enfermedad de Gaucher/metabolismo , Regulación hacia Abajo , Células Cultivadas , Supervivencia Celular , Encéfalo/metabolismo
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA