| Autor: |
Daniels LB, Glew RH, Radin NS, Vunnam RR |
| Jazyk: |
angličtina |
| Zdroj: |
Clinica chimica acta; international journal of clinical chemistry [Clin Chim Acta] 1980 Sep 25; Vol. 106 (2), pp. 155-63. |
| DOI: |
10.1016/0009-8981(80)90168-0 |
| Abstrakt: |
To date, enzymatic diagnosis of Gaucher's disease via a fluorometric assay procedure which utilizes 4-methylumbelliferyl-beta-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 beta-glucosidase and lysosomal glucocerebrosidase activities in crude extracts of liver. Incorporation of conduritol-beta-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 assy procedure which utilizes authentic, radiolabeled glucocerebroside as the substrate in demonstrating a deficiency of glucocerebrosidase activity in liver. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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