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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.

PMID:3134028
Abstract

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.

摘要

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。

相似文献

1
L-gulono-gamma-lactone oxidase deficiency in rats with osteogenic disorder: enzymological and immunochemical studies.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
Biochem Int. 1988 Apr;16(4):615-21.
2
Micro-determination of L-gulono-gamma-lactone oxidase activity.L-古洛糖酸-γ-内酯氧化酶活性的微量测定
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In vitro synthesis of L-gulono-gamma-lactone oxidase by rabbit reticulocyte lysate.兔网织红细胞裂解物体外合成L-古洛糖酸-γ-内酯氧化酶。
Biochem Int. 1987 Oct;15(4):779-83.
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A missense mutation of L-gulono-gamma-lactone oxidase causes the inability of scurvy-prone osteogenic disorder rats to synthesize L-ascorbic acid.L-古洛糖酸-γ-内酯氧化酶的错义突变导致易患坏血病的成骨障碍大鼠无法合成L-抗坏血酸。
J Biol Chem. 1992 Oct 25;267(30):21973-6.
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Transgenic expression of L-gulono-gamma-lactone oxidase in medaka (Oryzias latipes), a teleost fish that lacks this enzyme necessary for L-ascorbic acid biosynthesis.将L-古洛糖酸-γ-内酯氧化酶在青鳉(一种缺乏L-抗坏血酸生物合成所需这种酶的硬骨鱼)中进行转基因表达。
Biochem Biophys Res Commun. 1996 Jun 25;223(3):650-3. doi: 10.1006/bbrc.1996.0949.
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Functional rescue of vitamin C synthesis deficiency in human cells using adenoviral-based expression of murine l-gulono-gamma-lactone oxidase.利用基于腺病毒的小鼠L-古洛糖酸-γ-内酯氧化酶表达对人类细胞中维生素C合成缺陷进行功能挽救。
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Production by a baculovirus expression system of the APO-protein of L-gulono-gamma-lactone oxidase, a flavoenzyme possessing a covalently-bound FAD.杆状病毒表达系统生产L-古洛糖酸-γ-内酯氧化酶的脱辅基蛋白,L-古洛糖酸-γ-内酯氧化酶是一种具有共价结合黄素腺嘌呤二核苷酸的黄素酶。
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Expression of the mutant gene for L-gulono-gamma-lactone oxidase in scurvy-prone rats.易患坏血病大鼠中L-古洛糖酸-γ-内酯氧化酶突变基因的表达
Experientia. 1989 Feb 15;45(2):126-9. doi: 10.1007/BF01954844.
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Effects of fetal liver transplantation in congenitally enzyme deficient rats.胎肝移植对先天性酶缺乏大鼠的影响。
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Molecular basis for the deficiency in humans of gulonolactone oxidase, a key enzyme for ascorbic acid biosynthesis.人类中缺乏古洛糖酸内酯氧化酶(抗坏血酸生物合成的关键酶)的分子基础。
Am J Clin Nutr. 1991 Dec;54(6 Suppl):1203S-1208S. doi: 10.1093/ajcn/54.6.1203s.

引用本文的文献

1
Senescence marker protein 30 functions as gluconolactonase in L-ascorbic acid biosynthesis, and its knockout mice are prone to scurvy.衰老标记蛋白30在L-抗坏血酸生物合成中作为葡糖酸内酯酶发挥作用,其基因敲除小鼠易患坏血病。
Proc Natl Acad Sci U S A. 2006 Apr 11;103(15):5723-8. doi: 10.1073/pnas.0511225103. Epub 2006 Apr 3.
2
Expression of the mutant gene for L-gulono-gamma-lactone oxidase in scurvy-prone rats.易患坏血病大鼠中L-古洛糖酸-γ-内酯氧化酶突变基因的表达
Experientia. 1989 Feb 15;45(2):126-9. doi: 10.1007/BF01954844.