用环胞菌素A建立EB病毒转化的人类若干遗传病的淋巴母细胞系

Establishment of EBV-transformed lymph-oblastoid cell lines of several human genetic diseases with cyclosporin A

免疫抑制剂环胞菌素Ａ（ＣｙＡ），是一种真菌代谢物，能够选择性地抑制ＴＬＣ，防止ＢＬＣ的退化，将其引进ＥＢＶ转化的淋巴母细胞的建系过程，能够很大地提高建系的成功率，也是方法学上的一大突破。本研究采用ＣｙＡ，建立了ＥＢＶ转化的人类若干遗传病（共济失调４例、Ｗｉｌｓｏｎ氏病３例、Ｙｑ－综合征２例）、正常群体２０余株的淋巴母细胞系，为人类的医学遗传研究提供了一个库存基地。

Cyclosporin A(CyA),fungal metabolite and immunosuppressive agent,can specifically inhibit TLC and protect BLC from regression.The introduction of CyA in establishing EBV-transformed human LCL is a methodological breakthrough and increases the success rate of the establishment remarkably.By using CyA,We have established EBV-transformed LCL of several human genetic diseases(9 cases)and more than 20 healthy individuals as well.Among the 9 cases.4 suffered with ataxia,2 with wilson's disease and 2 with Yq-syndrome.This bank provides a source for human medical genetic investigation.