DIAGNOSIS OF GAUCHERS-DISEASE IN LIVER USING 4-METHYLUMBELLIFERYL-BETA-D-GLUCOPYRANOSIDE
DIAGNOSIS OF GAUCHERS-DISEASE IN LIVER USING 4-METHYLUMBELLIFERYL-BETA-D-GLUCOPYRANOSIDE
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DOI：
10.1016/0009-8981(77)90512-5
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发表时间：
1977-01-01
影响因子：
5
通讯作者：
BUTTERWORTH, J
中科院分区：
文献类型：
--
作者：
BROADHEAD, DM;BUTTERWORTH, J
Cases of Gaucher''s disease (human) could not be distinguished from controls by the assay of .beta.-glucosidase activity in H2O homogenates of liver using 4-methylumbelliferyl-.beta.-D-glucopyranoside. 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. 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 NaCl, a component of the elution buffer.
