Turnover of beta-galactosidase in fibroblasts from patients with genetically different types of beta-galactosidase deficiency.

The turnover of lysosomal beta-galactosidase was studied in fibroblast cultures from patients with Gm1-gangliosidosis and combined beta-galactosidase and neuraminidase deficiency, which had 5-10% residual beta-galactosidase activity. beta-Galactosidase was specifically inactivated with the suicide s...

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Hauptverfasser: Van Diggelen, O P, Schram, A W, Sinnott, M L, Smith, P J, Robinson, D, Galjaard, H
Format: Artigo
Sprache:Inglês
Veröffentlicht: 1981
Schlagworte:
Research Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1163512/
https://ncbi.nlm.nih.gov/pubmed/6800355
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