狐松鼠（Sciurus niger）中的尿卟啉原3合酶活性。

Uroporphyrinogen 3 cosynthetase activity in the fox squirrel (Sciurus niger).

作者信息

Levin E Y, Flyger V

出版信息

Science. 1971 Oct 1;174(4004):59-60. doi: 10.1126/science.174.4004.59.

DOI:10.1126/science.174.4004.59

PMID:5120867

Abstract

The activity of uroporphyrinogen III cosynthetase in hemolyzates and tissue extracts from fox squirrels is much less than in similar preparations from gray squirrels. Low activity of this enzyme explains the production of large amounts of uroporphyrin I by the fox squirrel. Members of this species thus provide a small-animal model for studies of congenital erythropoietic porphyria, a hereditary disease of man and cattle which is associated with a similar partial deficiency of uroporphyrinogen III cosynthetase.

摘要

狐松鼠溶血产物和组织提取物中尿卟啉原III同合成酶的活性远低于灰松鼠的类似提取物。该酶活性低解释了狐松鼠产生大量尿卟啉I的原因。因此，该物种成员为先天性红细胞生成性卟啉病的研究提供了一个小动物模型，先天性红细胞生成性卟啉病是人类和牛的一种遗传性疾病，与尿卟啉原III同合成酶的类似部分缺乏有关。