Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice

II3NeuAc-GgOse4Cer (GM1) gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid β-galactosidase (β-gal), resulting in the accumulation of ganglioside GM1 and its asialo derivative GgOse4Cer (GA1) in the central nervous system, primarily in the brain. In this study, w...

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Auteurs principaux:	J.L. Kasperzyk, A. d'Azzo, F.M. Platt, J. Alroy, T.N. Seyfried
Format:	Artigo
Langue:	Inglês
Publié:	Elsevier 2005-04-01
Collection:	Journal of Lipid Research
Sujets:	N-butyldeoxygalactonojirimycin II3 (NeuAc)2-LacCer II3NeuAc-GgOse4Cer asialo derivative myelin cerebrosides
Accès en ligne:	http://www.sciencedirect.com/science/article/pii/S0022227520340098
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