Multiple abnormalities of ectoglycosyltransferases in cultured fibroblasts from patients with mucolipidosis II: Possible indication for abnormal plasma membrane glycoproteins. View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1977-10

AUTHORS

STEFANO DI DONATO, ULRICH N. WIESMANN, E. ROSSI, NORBERT HERSCHKOWITZ

ABSTRACT

Ectoglycosyltransferase activities were measured in cultured normal and mucolipidosis II fibroblasts using endogenous glycoproteins and glycolipids and whole cells. Nucleotide pyrophosphatase, known to interfere with glycosyltransferases, was completely inhibited with 6 mM 5' AMP. Since preliminary experiments have shown multiple abnormalities of ectoglycosyltransferases in mucolipidosis II fibroblasts (Di Donato, unpublished results), galactosyl (Gal)-and N-acetylglucosaminyl (GluNac)-transferase were studied in further detail in confluent and nonconfluent cultures of normal and patient fibroblasts. Activities of both transferases on glycoproteins were higher in nonconfluent cultures. A 50% reduced activity of Gal-transferase was measured in confluent mucolipidosis II cultures and of GluNac-transferase in nonconfluent mucolipidosis II cultures towards incorporation of sugar precursors into glycoproteins. Substrate saturation kinetics of both transferases in mucolipidosis II fibroblasts revealed an abnormal Km for Gal incorporation into endogenous glycoproteins. Glycosylation of glycolipids in mucolipidosis fibroblasts was normal. More... »

PAGES

1094-6

Identifiers

URI

http://scigraph.springernature.com/pub.10.1203/00006450-197711100-00006

DOI

http://dx.doi.org/10.1203/00006450-197711100-00006

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1064205687

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/904973


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