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Ganglioside GM2 N-acetyl-beta-D-galactosaminidase activity in cultured fibroblasts of late-infantile and adult GM2 gangliosidosis patients and of healthy probands with low hexosaminidase level.

A sensitive assay was developed to assess the ability of extracts from cultured fibroblasts to catabolize ganglioside GM2, in the presence of the natural activator protein but without detergents. This method, which permitted the reliable determination of residual activities as low as 0.1% of normal...

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Detalhes bibliográficos
Main Authors: Conzelmann, E, Kytzia, H J, Navon, R, Sandhoff, K
Formato: Artigo
Idioma:Inglês
Publicado em: 1983
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1685812/
https://ncbi.nlm.nih.gov/pubmed/6614006
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