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  • Title: A microassay for acid beta-galactosidase activity toward asialofetuin.
    Author: Mutoh T, Naoi M, Sobue I, Kiuchi K, Nagatsu T.
    Journal: Clin Chim Acta; 1985 Nov 15; 152(3):307-14. PubMed ID: 2415273.
    Abstract:
    To study the enzymatic properties of beta-galactosidase from the patients with a beta-galactosidase deficiency such as GM1 gangliosidosis, determination of enzymatic activity with naturally occurring substrates, asialofetuin in addition to another natural substrate, GM1 ganglioside, is essentially required. With a previously reported, simple and sensitive fluorometric assay for GM1 ganglioside beta-galactosidase using high performance liquid chromatography (HPLC), optimal reaction conditions were determined for the assay of acid beta-galactosidase activity toward asialofetuin in skin fibroblast homogenates. Under these conditions, reduced enzymatic activities could be detected in cultured skin fibroblasts from patients with type 1 and 3 GM1 gangliosidoses and mucopolysaccharidosis IV-B (Morquio B syndrome). This method was applicable to study of the enzymatic properties of the mutant beta-galactosidase and provided an alternative to assays employing radioactive or artificial substrates.
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