A revised fluorometric assay for Gaucher's disease using conduritol-beta-epoxide with liver as the source of Beta-glucosidase
| Autor: | Lydia B. Daniels, Norman S. Radin, Ranga R. Vunnam, Robert H. Glew |
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| Rok vydání: | 1980 |
| Předmět: |
Clinical Biochemistry
Epoxide Glucocerebroside Biochemistry chemistry.chemical_compound medicine Humans Fluorometry Beta (finance) chemistry.chemical_classification Gaucher Disease Beta-glucosidase beta-Glucosidase Biochemistry (medical) General Medicine Clinical Enzyme Tests medicine.disease Lysosomal Glucocerebrosidase Enzyme Gaucher's disease chemistry Liver Glucosylceramidase Glucosidases Inositol Conduritol |
| Zdroj: | Clinica chimica acta; international journal of clinical chemistry. 106(2) |
| ISSN: | 0009-8981 |
| Popis: | To date, enzymatic diagnosis of Gaucher's disease via a fluorometric assay procedure which utilizes 4-methylumbelliferyl-β- d -glucopyranoside as a substrate has not been possible when liver serves as the source of enzyme since currently employed fluorometric procedures cannot adequately differentiate between a broad-specificity β-glucosidase and lysosomal glucocerebrosidase activities in crude extracts of liver. Incorporation of conduritol-β-epoxide into the incubation medium for the fluorometric assay allows one to selectively measure the glucocerebrosidase activity present in a given liver extract. In five cases of Gaucher's disease this revised fluorometric procedure proved as effective as the assay procedure which utilizes authentic, radiolabeled glucocerebroside as the substrate in demonstrating a deficiency of glucocerebrosidase activity in liver. |
| Databáze: | OpenAIRE |
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