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Differential assay for lysosomal alpha-galactosidases in human tissues and its application to Fabry's disease.

Authors
Type
Published Article
Journal
Clinica Chimica Acta
0009-8981
Publisher
Elsevier
Publication Date
Volume
112
Issue
2
Pages
247–251
Identifiers
PMID: 6263521
Source
Medline

Abstract

A simple and sensitive fluorometric method has been described for the differential determination of the activity of lysosomal alpha-galactosidase A and alpha-galactosidase B. The procedure employs 4-methylumbelliferyl-alpha-D-galactopyranoside as substrate and N-acetylgalactosamine as an inhibitor of alpha-galactosidase B, but not of alpha-galactosidase A to differentiate the two activities. This method was shown to be applicable in the differentiation of the two enzyme activities in human tissues and in the diagnosis of the heterozygous and hemizygous genotypes for Fabry's disease in cultured skin fibroblasts.

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