| Literature DB >> 812485 |
G Romeo, M D'Urso, A Pisacane, E Blum, A De Falco, A Ruffilli.
Abstract
The alpha-galactosidase A activity from fibroblasts of five Fabry patients and five controls has been separated from alpha-galactosidase B through small DEAE-cellulose columns and in some experiments by treatment of the fibroblast extracts with Sepharose coupled to anti-alpha-galactosidase B antibodies. By these independent methods, it has been shown that there is a residual alpha-galactosidase A in Fabry's disease, which is immunologically similar to the alpha-galactosidase A from the controls. The alpha-galactosidase A from all of the patients and controls has the same apparent Km value for the synthetic substrate 4-methylumbelliferyl-alpha-galactosidase A, while the fifth has a thermolabile enzyme like that from the controls. The amount of immunologically active alpha-galactosidase A seems to be decreased in the patients tested.Entities:
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Year: 1975 PMID： 812485 DOI： 10.1007/BF00484919
Source DB: PubMed Journal: Biochem Genet ISSN： 0006-2928 Impact factor: 1.890