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腺苷脱氨酶缺乏症中血小板内dATP的积累和ATP的消耗:对免疫反应有何意义?

dATP accumulation and ATP depletion in platelets in adenosine deaminase deficiency: significance for the immune response?

作者信息

Simmonds H A, Goday A, Morris G S, Fairbanks L D, Levinsky R J

出版信息

Biosci Rep. 1984 Oct;4(10):809-18. doi: 10.1007/BF01138162.

Abstract

High levels of dATP and dADP, accompanied by ATP depletion, were found in the platelets of two ADA-deficient children with severe combined immunodeficiency (SCID). In vitro studies demonstrated that even normal platelets had the ability to make dATP from deoxyadenosine (dAR) under physiological conditions. This capability was greatly enhanced by conditions simulating ADA deficiency. These results question whether the platelet has a specific role in the normal immune response.

摘要

在两名患有严重联合免疫缺陷(SCID)的腺苷脱氨酶(ADA)缺乏症儿童的血小板中,发现了高水平的脱氧三磷酸腺苷(dATP)和脱氧二磷酸腺苷(dADP),同时伴有三磷酸腺苷(ATP)耗竭。体外研究表明,即使是正常血小板在生理条件下也有能力从脱氧腺苷(dAR)生成dATP。模拟ADA缺乏的条件极大地增强了这种能力。这些结果使人质疑血小板在正常免疫反应中是否具有特定作用。

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