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Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1971 Nov;68(11):2810–2813. doi: 10.1073/pnas.68.11.2810

Gaucher's Disease: Deficiency of “cid”β-Glucosidase and Reconstitution of Enzyme Activity In Vitro

Mae Wan Ho 1, John S O'Brien 1
PMCID: PMC389531  PMID: 5288260

Abstract

The spleen from a patient with adult Gaucher's disease was shown to be deficient in a β-glucosidase (EC 3.2.1.21) isoenzyme that has optimal activity at pH 4.0-4.3, and is stimulated by 0.02% Triton X-100. A mixture of spleen homogenates from a control and from the patient contained β-glucosidase activity equivalent to 2-3 times the theoretical expected activity. The increase in enzyme activity occurred at pH 4.0-4.3; the magnitude of the increase was proportional to the amount of each homogenate added. Two factors, one called factor P from the patient's spleen, the other called factor C from the control spleen, were responsible for a reconstitution of β-glucosidase activity in vitro. Factor P is tentatively identified as an acid glycoprotein.

Keywords: spleen, components of enzyme

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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