突变小鼠中缺乏腺苷脱氨酶导致消瘦。

Lack of adenosine deaminase deficiency in the mutant mouse wasted.

作者信息

Geiger J D, Nagy J I

出版信息

FEBS Lett. 1986 Nov 24;208(2):431-4. doi: 10.1016/0014-5793(86)81063-8.

DOI:10.1016/0014-5793(86)81063-8

Abstract

The possibility that the mutant mouse wasted (wst/wst) may serve as an animal model for studies of severe combined immunodeficiency disease (SCID) and the role of adenosine deaminase (ADA, EC 3.5.4.4) in adenosine metabolism were investigated. The specific activity of ADA in wst/wst compared with control mice was significantly lower by 26% in thymus, but significantly higher by 18% in spleen and 32% in cerebellum. Vmax values of ADA in spleens were 43% higher in wst/wst mice and no changes were observed in Km values. In contrast, the Vmax of ADA was unchanged in erythrocytes from wst/wst mice, but the Km for adenosine was significantly elevated. Thus, based on ADA measurements alone, it may be premature to consider wst/wst mice as a model for ADA deficiency and SCID in humans.

摘要

研究了突变型小鼠wasted（wst/wst）作为严重联合免疫缺陷病（SCID）研究的动物模型以及腺苷脱氨酶（ADA，EC 3.5.4.4）在腺苷代谢中的作用的可能性。与对照小鼠相比，wst/wst小鼠胸腺中ADA的比活性显著降低26%，但脾脏中显著升高18%，小脑则升高32%。wst/wst小鼠脾脏中ADA的Vmax值高43%，Km值未观察到变化。相反，wst/wst小鼠红细胞中ADA的Vmax未改变，但腺苷的Km值显著升高。因此，仅基于ADA测量结果，将wst/wst小鼠视为人类ADA缺乏和SCID的模型可能为时过早。

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引用本文的文献

1

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Biochem Genet. 1991 Dec;29(11-12):537-44. doi: 10.1007/BF02426869.

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