Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD)

Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD)

The twitcher mouse is a pathologically and enzymatically authentic model of globoid cell leukodystrophy (GLD, Krabbe disease) that has been widely used for the evaluation of potential therapeutic approaches. This naturally occurring mouse model contains a premature stop codon (W339X) in the galactos...

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Bibliographic Details
Main Authors: Wing C. Lee, Yuen K. Tsoi, Chad A. Dickey, Michael W. DeLucia, Dennis W. Dickson, Christopher B. Eckman
Format: Article
Language:English
Published: Elsevier 2006-08-01
Series:Neurobiology of Disease
Online Access:http://www.sciencedirect.com/science/article/pii/S096999610600057X

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