成骨障碍大鼠中L-古洛糖酸-γ-内酯氧化酶缺乏：酶学和免疫化学研究
L-gulono-gamma-lactone oxidase deficiency in rats with osteogenic disorder: enzymological and immunochemical studies.
作者信息
Nishikimi M, Koshizaka T, Mochizuki H, Iwata H, Makino S, Hayashi Y, Ozawa T, Yagi K
机构信息
Department of Biomedical Chemistry, Faculty of Medicine, University of Nagoya, Japan.
出版信息
Biochem Int. 1988 Apr;16(4):615-21.
A strain of Wistar rats with L-gulono-gamma-lactone oxidase deficiency has recently been established. The activity of L-gulono-gamma-lactone oxidase in hepatic microsomes of the mutant rats was not detectable, while that of heterozygous rats was about half that of normal rats. These results were confirmed by immunological quantitation using antibody directed against this enzyme. Thus, it appears that these mutant rats either possess no enzyme at all or a very aberrant form of the enzyme in the liver. Aldonolactonase, another enzyme participating in the biosynthesis of L-ascorbic acid, was found to be present in normal amount in the mutant strain.
最近培育出了一种缺乏L-古洛糖酸-γ-内酯氧化酶的Wistar大鼠品系。突变大鼠肝脏微粒体中L-古洛糖酸-γ-内酯氧化酶的活性无法检测到，而异合子大鼠的该酶活性约为正常大鼠的一半。使用针对该酶的抗体进行免疫定量分析证实了这些结果。因此，这些突变大鼠似乎在肝脏中要么完全没有这种酶，要么拥有一种非常异常形式的该酶。参与L-抗坏血酸生物合成的另一种酶醛糖内酯酶，在突变品系中的含量正常。
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