THE OCCURRENCE OF 2 IMMUNOLOGICALLY DISTINGUISHABLE BETA-GLUCOCEREBROSIDASES IN HUMAN SPLEEN
THE OCCURRENCE OF 2 IMMUNOLOGICALLY DISTINGUISHABLE BETA-GLUCOCEREBROSIDASES IN HUMAN SPLEEN
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DOI：
10.1111/j.1432-1033.1985.tb09058.x
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发表时间：
1985-01-01
期刊：
影响因子：
--
通讯作者：
SCHRAM, AW
中科院分区：
文献类型：
--
作者：
AERTS, JMFG;DONKERKOOPMAN, WE;SCHRAM, AW
The .beta.-glucosidase activity in spleen from control subjects and patients with different clinical phenotypes of Gaucher''s disease was characterized. The occurrence of a soluble non-specific .beta.-glucosidase with a neutral pH optimum and 2 membrane-associated .beta.-glucocerebrosidases with an acid pH optimum was demonstrated. The 2 .beta.-glucocerebrosidases can be distinguished on the basis of their ability to react with anti-(placental .beta.-glucocerebrosidase) antibodies bound to protein-A-Sepharose 4B beads. One of the splenic .beta.-glucocerebrosidases (form I) is precipitated by the immobilized antibodies and the other (form II) is not. The 2 forms also differ in binding affinity to concanavalin A, degree of stimulation of enzymic activity by taurocholate and isoelectric point. The Km values of the 2 .beta.-glucocerebrosidases for natural and artificial substrates are similar and both are inhibited by conduritol B-epoxide. In spleen from 3 patients with type 1, 1 patient with type 2 and 1 patient with type 3 Gaucher''s disease form I .beta.-glucocerebrosidase was found to be clearly deficient; the activity of form II was 25-50% of that in control spleen. The non-specific, neutral .beta.-glucosidase was not deficient in these Gaucher spleens. The distinct biochemical and immunological properties of non-specific .beta.-glucosidase and the fact that normal levels of the enzyme are present in patients with Gaucher''s disease indicate, in confirmation of previous reports, that non-specific .beta.-glucosidase is not related to .beta.-glucocerebrosidase.
