Background: The concentration and specific activity of N-acetyl-b-hexosaminidase (HEX) in palatine tonsils with chronic tonsillitis and tonsillar hypertrophy give insight in tonsillar tissue remodeling and constitute a potential marker for diagnosis and treatment of chronic tonsillitis and tonsillar hypertrophy. Aim: Determining the concentration and specific activity of N-acetyl-b-hexosaminida...

Previous studies of the subunit structure of hexosaminidase gave ambiguous results, but suggested that the enzyme was composed of six equally sized subunits. Dissociation of hexosaminidase A with p-chloromercuribenzoate produces an alkylated fragment with mol.wt. approx. 50000, which is converted into hexosaminidase S by treatment with dithiothreitol. Treatment ofnative hexosaminidase A with so...

rendered background IgE levels only. In accordance, sera of OVA mice which permitted mast cell degranulation upon OVA trigger in a specific b-hexosaminidase release assay, whereas sera of OVA-AAVLP mice did not contain anaphylactogenic antibodies. In an in vivo anaphylaxis experiment, upon intravenous OVA challenge OVA-immunized mice presented significant drop of body temperature, whereas AAVLP...

rendered background IgE levels only. In accordance, sera of OVA mice which permitted mast cell degranulation upon OVA trigger in a specific b-hexosaminidase release assay, whereas sera of OVA-AAVLP mice did not contain anaphylactogenic antibodies. In an in vivo anaphylaxis experiment, upon intravenous OVA challenge OVA-immunized mice presented significant drop of body temperature, whereas AAVLP...

The carboxylic ionophore, monensin, blocks the migration of glycoprotein-containing vesicles from the Golgi region to the plasma membrane in fibroblasts resulting in an accumulation of secretory products in the Golgi cisternae. Treatment of cultured I-cell fibroblasts with monensin (0.5,UM) decreased the abnormal excretion of f-hexosaminidase to 40% of untreated cultures within 15 min. A corres...

β-Hexosaminidase, which is generally present in the lysosome, is essential for glycoprotein metabolism in the maintenance of cell homeostasis. In mast cells (MCs), large amounts of β-hexosaminidase are present in the granules as opposed to the lysosome, and the biological role of MC β-hexosaminidase has yet to be fully elucidated. Therefore, we investigated the biological role of β-hexosaminida...

For a number of lysosomal hydrolases, abnormally expressed forms have been found to occur in childhood and adult leukemia. Most of these abnormalities are confined to the physicochemical properties of the enzymes, e.g., abnormal electrophoretic mobility, change of isoelectric point, and alteration of isoenzyme pattern. All this has been described for the hexosaminidase system in human leukemia ...

Variant AB of infantile GM2 gangliosidosis is a fatal disease leading invariably to death within the first few years of life, due to the excessive storage of the glycolipids GM2 and GA2 which occurs in the nervous tissue of the patient. Unlike other variants of this hereditary disease, where a deficiency of hexosaminidase A, the ganglioside-GM2-degrading enzyme, could be demonstrated, the varia...