English   español  
Por favor, use este identificador para citar o enlazar a este item: http://hdl.handle.net/10261/8747
Compartir / Impacto:
Estadísticas
Add this article to your Mendeley library MendeleyBASE
Citado 30 veces en Web of Knowledge®  |  Pub MebCentral Ver citas en PubMed Central  |  Ver citas en Google académico
Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL
Exportar otros formatos: Exportar EndNote (RIS)Exportar EndNote (RIS)Exportar EndNote (RIS)
Título

Ectopic expression of tyrosine hydroxylase in the pigmented epithelium rescues the retinal abnormalities and visual function common in albinos in the absence of melanin

Autor Lavado, Alfonso J.; Jeffery, Glen; Tovar, Victoria; Villa, Pedro de la; Montoliu, Lluís
Palabras clave Albinism
Chiasmatic projections
l-3,4-dihydroxyphenylalanine
Mitosis
Tyrosinase
Vision
Fecha de publicación 17-ene-2006
EditorJohn Wiley & Sons
Citación J Neurochem. 96(4): 1201-1211 (2006)
ResumenAlbino mammals have profound retinal abnormalities, including photoreceptor deficits and misrouted hemispheric pathways into the brain, demonstrating that melanin or its precursors are required for normal retinal development. Tyrosinase, the primary enzyme in melanin synthesis commonly mutated in albinism, oxidizes l-tyrosine to l-dopaquinone using l-3,4-dihydroxyphenylalanine (L-DOPA) as an intermediate product. L-DOPA is known to signal cell cycle exit during retinal development and plays an important role in the regulation of retinal development. Here, we have mimicked L-DOPA production by ectopically expressing tyrosine hydroxylase in mouse albino retinal pigment epithelium cells. Tyrosine hydroxylase can only oxidize l-tyrosine to L-DOPA without further progression towards melanin. The resulting transgenic animals remain phenotypically albino, but their visual abnormalities are corrected, with normal photoreceptor numbers and hemispheric pathways and improved visual function, assessed by an increase of spatial acuity. Our results demonstrate definitively that only early melanin precursors, L-DOPA or its metabolic derivatives, are vital in the appropriate development of mammalian retinae. They further highlight the value of substituting independent but biochemically related enzymes to overcome developmental abnormalities.
Descripción 11 pages, 5 figures.-- PMID: 16445854 [PubMed].-- Printed version published on Feb 2006.-- Additional material: CSIC Press release (Spanish).
Versión del editorhttp://dx.doi.org/10.1111/j.1471-4159.2006.03657.x
URI http://hdl.handle.net/10261/8747
DOI10.1111/j.1471-4159.2006.03657.x
ISSN0022-3042
Aparece en las colecciones: (CNB) Artículos
Ficheros en este ítem:
No hay ficheros asociados a este ítem.
Mostrar el registro completo
 



NOTA: Los ítems de Digital.CSIC están protegidos por copyright, con todos los derechos reservados, a menos que se indique lo contrario.