Radioimmunoassay determination of decreased amounts of alpha-L-fucosidase protein in fucosidosis.
Publication/Presentation Date
3-15-1982
Abstract
Purified human liver alpha-L-fucosidase (EC 3.2.1.51) has been radioiodinated by a chloramine-T procedure to a specific activity of 3.7 . 10(6) dpm/micrograms protein without altering its apparent Michaelis constant for the 4-methylumbelliferyl substrate. This 125I-labeled alpha-L-fucosidase has been used in development of a competitive binding radioimmunoassay for alpha-L-fucosidase which can detect 1-2 ng of enzyme protein and has been employed to quantify the amount of alpha-L-fucosidase protein in the liver and spleen from a patient with fucosidosis. Less than 1% of the normal amount of alpha-L-fucosidase protein is present suggesting that normal amounts of catalytically inactive alpha-L-fucosidase are not found in this disease.
Volume
715
Issue
1
First Page
90
Last Page
96
ISSN
0006-3002
Published In/Presented At
Andrews-Smith, G. L., & Alhadeff, J. A. (1982). Radioimmunoassay determination of decreased amounts of alpha-L-fucosidase protein in fucosidosis. Biochimica et biophysica acta, 715(1), 90–96. https://doi.org/10.1016/0304-4165(82)90053-8
Disciplines
Medicine and Health Sciences
PubMedID
7074133
Department(s)
Department of Medicine
Document Type
Article