The diagnosis of Gaucher's disease in liver using 4-methylumbelliferyl-beta-D-glucopyranoside.

Abstract

1. Cases of Gaucher's disease could not be distinguished from controls by the assay of beta-glucosidase activity in water homogenates of liver using 4-methylumbelliferyl-beta-D glucopyranoside. 2. Two peaks of beta-glucosidase activity were separated by Sephadex G-150 gel filtration in control and Gaucher livers. In the presence of the elution buffer pH profiles of peak I showed a deficiency at pH 3.5-4.5 in Gaucher's disease. Gaucher and control peak II had similar pH profiles with little or no activity at pH 3.0-4.0. 3. A clear distinction between homogenates of Gaucher and control liver was obtained by assay at pH 4.0 in the presence of elution buffer, or of sodium chloride, a component of the elution buffer.

• Authors: D M Broadhead, J Butterworth
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 75 Issue 1 Pg. 155-61 (Feb 15 1977) ISSN: 0009-8981 [Print] Netherlands
• PMID: 14796 (Publication Type: Journal Article)

Chemical References

• Glucosides
• Umbelliferones
• 4-methylumbelliferyl glucoside
• Hymecromone
• Glucosidases
• Glucuronidase

Topics

• Gaucher Disease (diagnosis)
• Glucosidases (analysis)
• Glucosides
• Glucuronidase (analysis)
• Humans
• Hydrogen-Ion Concentration
• Hymecromone (analogs & derivatives)
• Liver (enzymology)
• Umbelliferones