Compartmental distribution of β-hexosaminidase isoenzymes in I-cell fibroblasts
نویسندگان
چکیده
منابع مشابه
P-hexosaminidase transport in normal and I-cell fibroblasts
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...
متن کاملExcretion-reuptake route of beta-hexosaminidase in normal and I-cell disease cultured fibroblasts.
It has been proposed that in cultured fibroblasts the final packaging of enzymes in lysosomes requires excretion followed by pinocytosis by neighboring cells via a carbohydrate-specific receptor mechanism. It has also been proposed that the abnormally high activity of lysosomal enzymes in the medium of cultured fibroblasts from patients with I-cell disease (mucolipidosis II) results from an alt...
متن کاملMonocyte Secretion of β-Hexosaminidase in Patients With Obstructive Jaundice
Monocyte hydrolases are harmful when secreted inappropriately. In this study we have investigated the levels of one of the hydrolases. beta-hexosaminidase in patients with obstructive jaundice. These patients showed markedly elevated plasma levels, and their monocytes show increased spontaneous secretion and total enzyme content. The plasma enzyme levels correlate with monocyte enzyme content a...
متن کاملDoes β-hexosaminidase function only as a degranulation indicator in mast cells? The primary role of β-hexosaminidase in mast cell granules.
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...
متن کاملRole of N-acetyl-β-d-hexosaminidase in cholesteatoma tissue
Cholesteatoma is a destructive disease characterized by the progressive expansion of keratinizing squamous epithelium in the middle ear and mastoid, and chronic inflammatory reaction of the subepithelial connective tissue. N-Acetyl-β-d-hexosaminidase (HEX) catalyzes the release of terminal non-reducing N-acetyl-d-hexosamine residues acting on glucosides and galactosides in glycoproteins, GM2-ga...
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ژورنال
عنوان ژورنال: Biochemical Journal
سال: 1981
ISSN: 0264-6021
DOI: 10.1042/bj1960657