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
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
收藏
页码:841 / 849
页数:9
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