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Variant of GM2-gangliosidosis with hexosaminidase A having a severely changed substrate specificity.

The levels of hexosaminidase A activity in cultivated fibroblasts of two patients with GM2-gangliosidosis were close to the normal range with 4-methylumbelliferyl-beta-D-2-acetamido-2-deoxyglucopyranoside and 4-methylumbelliferyl-beta-D-2-acetamido-2-deoxygalactopyranoside as substrates, and the enz...

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Detalhes bibliográficos
Main Authors: Kytzia, H J, Hinrichs, U, Maire, I, Suzuki, K, Sandhoff, K
Formato: Artigo
Idioma:Inglês
Publicado em: 1983
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC555256/
https://ncbi.nlm.nih.gov/pubmed/6226523
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