COMPARED EXPRESSION LEVELS OF ORNITHINE TRANSCARBAMYLASE AND CARBAMYLPHOSPHATE SYNTHETASE IN LIVER AND SMALL-INTESTINE OF NORMAL AND MUTANT MICE
COMPARED EXPRESSION LEVELS OF ORNITHINE TRANSCARBAMYLASE AND CARBAMYLPHOSPHATE SYNTHETASE IN LIVER AND SMALL-INTESTINE OF NORMAL AND MUTANT MICE
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DOI：
10.1016/0167-4781(88)90128-5
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发表时间：
1988-09-07
期刊：
影响因子：
--
通讯作者：
BRIAND, P
中科院分区：
文献类型：
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作者：
DUBOIS, N;CAVARD, C;BRIAND, P
采用酶法、电泳免疫印迹和RNA点印迹技术研究了鸟氨酸转甲氨基酰基酶（OTC）基因在皮肤毛发异常的稀疏毛小鼠（Spf）和x连锁OTC缺乏的稀疏毛小鼠（Spf）肝脏和小肠中的表达。我们在这两种组织中发现OTC活性降低。我们现在表明，这种减少在肠道比在肝脏Spf-ash菌株更不明显。在出生后的前两周，这种缺陷似乎比成年小鼠要少。尿素循环的另一种酶氨甲酰磷酸合成酶I （CPS）的酶活性仅在Spf突变株中显著改变。
Enzymatic assay, electrophoretic immunoblotting and RNA dot-blot techniques were employed to investigate the expression of the ornithine transcarbamylase (OTC) gene in liver and small intestine of Sparse fur mice with abnormal skin and hair (Spf-ash) and Sparse fur mice (Spf) which exhibit an X-linked OTC deficiency. We found a reduced OTC activity in these two tissues. We now show that this reduction is less pronounced in the intestine than in the liver of the Spf-ash strain. During the first 2 weeks of life, the deficiency appears to be less than in the adult mice. The enzymatic activity of carbamylphosphate synthetase I (CPS), another enzyme of the urea cycle, is significantly modified in the Spf mutant strain only.