Arginine-related guanidino compounds and nitric oxide synthase in the brain of ornithine transcarbamylase deficient spf mutant mouse: Effect of metabolic arginine deficiency

被引：27

作者：

Ratnakumari, L

Qureshi, IA

Butterworth, RF

Marescau, B

DeDeyn, PP

机构：

[1] HOP ST JUSTINE,DIV MED GENET,MONTREAL,PQ H3T 1C5,CANADA

[2] ST LUC HOSP,NEUROSCI RES UNIT,MONTREAL,PQ,CANADA

[3] UNIV ANTWERP,UIA,BORN BUNGE FDN,LAB NEUROCHEM & BEHAV,B-2610 ANTWERP,BELGIUM

来源：

NEUROSCIENCE LETTERS | 1996年 / 215卷 / 03期

关键词：

guanidines; brain; congenital hyperammonemia; nitric oxide synthase; metabolic arginine deficiency;

D O I：

10.1016/0304-3940(96)12975-X

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

The sparse-fur (spf) mouse, with an X-linked hepatic ornithine transcarbamylase (OTC, E.C.2.1.3.3) deficiency, exhibits significantly lower levels of arginine in the brain as compared to normal controls. In the present study, the effect of a sustained lower metabolic arginine was studied by measuring the levels of several arginine-related guanidino compounds in brain. The concentrations of gamma-guanidinobutyric acid (gamma-GBA), N-alpha-acetylarginine (N-alpha-AA), argininic acid (Arg-A), guanidinoacetic acid (GAA), and creatine were significantly lower in spf mice as compared to controls. Since arginine is the precursor for nitric oxide, we also measured the activity of nitric oxide synthase which was significantly reduced in cerebellum, striatum, hippocampus and cerebral cortex of spf mice, The changes seen in cerebral guanidino compound and nitric oxide metabolism of spf mice could be due to a sustained deficiency of arginine, caused by a metabolic block in the urea cycle.

引用

页码：153 / 156

页数：4

共 23 条

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