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Urea cycle disorder in C3H-H-2 degree mice with juvenile steatosis of viscera.

作者信息

Imamura Y, Saheki T, Arakawa H, Noda T, Koizumi T, Nikaido H, Hayakawa J

机构信息

First Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan.

出版信息

FEBS Lett. 1990 Jan 15;260(1):119-21. doi: 10.1016/0014-5793(90)80081-s.

DOI:10.1016/0014-5793(90)80081-s
PMID:2298291
Abstract

We determined the activities of urea cycle enzymes in the liver of C3H-H-2 degree-jsv mice. The activities of all urea cycle enzymes decreased in the latter period of lactation. The activities of carbamylphosphate synthetase and ornithine transcarbamylase in some affected mice were undetectable. On the other hand, the activities of enzymes other than urea cycle enzymes were normal. We consider that the decrease in the urea cycle enzymes is caused by an abnormality in the mechanism of gene expression.

摘要

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引用本文的文献

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2
Hyperammonemia in carnitine-deficient adult JVS mice used by starvation.饥饿诱导的肉碱缺乏成年JVS小鼠出现高氨血症。
Metab Brain Dis. 2002 Dec;17(4):359-66. doi: 10.1023/a:1021966020057.
3
Transcriptional regulation of genes for ornithine cycle enzymes.
鸟氨酸循环酶基因的转录调控
Biochem J. 1995 Dec 15;312 ( Pt 3)(Pt 3):649-59. doi: 10.1042/bj3120649.
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Mitochondrial abnormalities of muscle tissue in mice with juvenile visceral steatosis associated with systemic carnitine deficiency.与全身性肉碱缺乏相关的青少年内脏脂肪变性小鼠肌肉组织中的线粒体异常。
Virchows Arch. 1995;426(3):271-9. doi: 10.1007/BF00191365.