Title
<tex>$\alpha$</tex>-l-Fucosidase in human fibroblasts : I. The enzyme activity polymorphism <tex>$\alpha$</tex>-l-Fucosidase in human fibroblasts : I. The enzyme activity polymorphism
Author
Faculty/Department
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences. Pharmacy
Faculty of Medicine and Health Sciences
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
Publication type
article
Publication
New York ,
Subject
Chemistry
Biology
Human medicine
Source (journal)
Biochemical genetics. - New York
Volume/pages
30(1992) :3-4 , p. 131-141
ISSN
0006-2928
ISI
A1992HU28400003
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
Low plasma alpha-L-fucosidase activity is a recessive polymorphic trait observed in 8% of the normal population. The molecular basis of this polymorphism remains unclear and its expression is tissue specific. As the low-activity (variant) phenotype is expressed in vitro in cultured human fibroblasts, this cell type was chosen to study the enzyme activity polymorphism. Fibroblast cell lines derived from individuals with low plasma fucosidase activity (variants) have less than 30% of the fucosidase activity of fibroblast cell lines established from individuals with high plasma fucosidase activity (nonvariants). No qualitative differences in the synthesis, processing, and extracellular release of newly made alpha-L-fucosidase could be demonstrated among variant and nonvariant cell strains. Cells pulsed with H-3-leucine for 10 min produce a 51-kDa protein which is rapidly processed to a 55-kDa intermediate. The latter is converted to a mature 59-kDa intracellular and a 61-kDa extracellular end product, in both variant and nonvariant fibroblast cell lines. Variant and nonvariant fibroblast cell lines also release relatively equal amounts of fucosidase into the extracellular medium. Therefore, differences in processing or extracellular release of fucosidase between variants and nonvariants are not the basic mechanism of this tissue-specific activity polymorphism.
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