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

Bases de datos
Tipo del documento
País de afiliación
Intervalo de año de publicación
1.
Sci Rep ; 11(1): 11432, 2021 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-34075074

RESUMEN

Retinitis Pigmentosa (RP) is a mostly incurable inherited retinal degeneration affecting approximately 1 in 4000 individuals globally. The goal of this work was to identify drugs that can help patients suffering from the disease. To accomplish this, we screened drugs on a zebrafish autosomal dominant RP model. This model expresses a truncated human rhodopsin transgene (Q344X) causing significant rod degeneration by 7 days post-fertilization (dpf). Consequently, the larvae displayed a deficit in visual motor response (VMR) under scotopic condition. The diminished VMR was leveraged to screen an ENZO SCREEN-WELL REDOX library since oxidative stress is postulated to play a role in RP progression. Our screening identified a beta-blocker, carvedilol, that ameliorated the deficient VMR of the RP larvae and increased their rod number. Carvedilol may directly on rods as it affected the adrenergic pathway in the photoreceptor-like human Y79 cell line. Since carvedilol is an FDA-approved drug, our findings suggest that carvedilol can potentially be repurposed to treat autosomal dominant RP patients.


Asunto(s)
Animales Modificados Genéticamente , Conducta Animal/efectos de los fármacos , Enfermedades Genéticas Congénitas , Retinitis Pigmentosa , Rodopsina , Visión Ocular , Pez Cebra , Animales , Animales Modificados Genéticamente/genética , Animales Modificados Genéticamente/metabolismo , Línea Celular , Evaluación Preclínica de Medicamentos , Enfermedades Genéticas Congénitas/tratamiento farmacológico , Enfermedades Genéticas Congénitas/genética , Enfermedades Genéticas Congénitas/metabolismo , Humanos , Mutación , Células Fotorreceptoras Retinianas Bastones , Retinitis Pigmentosa/tratamiento farmacológico , Retinitis Pigmentosa/genética , Retinitis Pigmentosa/metabolismo , Rodopsina/genética , Rodopsina/metabolismo , Transgenes , Visión Ocular/efectos de los fármacos , Visión Ocular/inmunología , Pez Cebra/genética , Pez Cebra/metabolismo
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA