HIGH-FREQUENCY OF BETA-HEXOSAMINIDASE DEFICIENCY IN LYMPHOBLASTOID CELL-LINES

被引：8

作者：

DREYFUS, JC ^{[1
]}

BELON, JP ^{[1
]}

GAUTRON, S ^{[1
]}

LENOIR, G ^{[1
]}

POENARU, L ^{[1
]}

机构：

[1] INT AGCY RES CANC, F-69372 LYON 2, FRANCE

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1984年 / 119卷 / 03期

关键词：

D O I：

10.1016/0006-291X(84)90850-7

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Activity of 7 lysosomal enzymes was determined in 25 [human] lymphoblastoid cell lines. These lines included normal controls transformed with Epstein-Barr virus [EBr], Burkitt''s lymphomas and other lymphomas with or without EBV genome. Four lines were deficient in total .beta.-hexosaminidase activity. The deficiency was as severe as that of the variant O (Sandhoff''s disease) of clinical .beta.-hexosaminidase deficiency. The electrophoretic pattern was also similar to that observed in Sandhoff''s disease. Possible mechanisms explaining the high frequency of .beta.-hexosaminidase deficiency in lymphoblastoid cell lines are discussed.

引用

页码：841 / 849

页数：9

共 18 条

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