These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Beta-glucosidase activity in liver, spleen and brain in acute neuropathic Gaucher disease.
    Author: Takahashi T, Nishio H, Kodama S, Nakamura H.
    Journal: Brain Dev; 1990; 12(2):202-5. PubMed ID: 2113779.
    Abstract:
    We examined the property of beta-D-glucosidase in normal child liver, spleen and brain tissues and in tissues affected by Gaucher disease using two different kinds of synthetic substrates, 2-hexadecanoylamino-4-nitrophenyl-beta-D-glucopyranoside (HN Glc) and 4-methylumbelliferyl-beta-D-glucopyranoside (MU Glc). HN glucosidase activity was noted only in the particulate fraction of each organ tissues of the normal child, and it was deficient in the tissue affected by Gaucher disease. MU glucosidase activity in the whole homogenate and the soluble fraction showed normal levels for the liver and brain tissue of the Gaucher patient. However, MU glucosidase activity in the particulate fraction, at the vicinity of pH 4.5, for the liver, spleen and brain tissue was deficient in the affected patient. HN Glc was a very easy and useful substrate for the diagnosis of Gaucher disease, but it was not very sensitive. While MU Glc was very sensitive, it was necessary to remove the non-specific beta-D-glucosidase for the diagnosis of Gaucher disease.
    [Abstract] [Full Text] [Related] [New Search]