Iyer R K, Bando J M, Jenkinson C P, Vockley J G, Kim P S, Kern R M, Cederbaum S D, Grody W W
Department of Pathology, UCLA School of Medicine, California 90095, USA.
Mol Genet Metab. 1998 Mar;63(3):168-75. doi: 10.1006/mgme.1997.2669.
Two forms of arginase, both catalyzing the hydrolysis of arginine to ornithine and urea, are found in animals ranging from amphibians to mammals. In humans, inherited deficiency of hepatic or type I arginase results in hyperargininemia, a syndrome characterized by periodic episodes of hyperammonemia, spasticity, and neurological deterioration. In these patients, a second extrahepatic or type II arginase activity is significantly increased, an induction that may partially compensate for the lack of AI activity and apparently mitigates some of the clinical effects of the condition. Cloning and characterization of the human AII cDNA was recently accomplished. The cloning, sequencing, and partial characterization of the mouse and rat AII cDNAs are reported herein. The DNA sequences predicted polypeptides of 354 amino acids, including a N-terminal mitochondrial import signal. Sequence homology to the human type II arginase, arginase activity data, and immunoprecipitation with an anti-AII antibody confirm the identity of these cloned genes as rodent extrahepatic type II arginases.
从两栖动物到哺乳动物的各类动物体内均发现了两种精氨酸酶,它们都能催化精氨酸水解生成鸟氨酸和尿素。在人类中,肝脏型或I型精氨酸酶的遗传性缺乏会导致高精氨酸血症,该综合征的特征为周期性高氨血症发作、痉挛和神经功能恶化。在这些患者中,第二种肝外型或II型精氨酸酶活性显著增加,这种诱导作用可能部分补偿了I型精氨酸酶活性的缺乏,并明显减轻了该病症的一些临床症状。最近完成了人类II型精氨酸酶cDNA的克隆和特性分析。本文报道了小鼠和大鼠II型精氨酸酶cDNA的克隆、测序及部分特性分析。DNA序列预测的多肽由354个氨基酸组成,包括一个N端线粒体导入信号。与人类II型精氨酸酶的序列同源性、精氨酸酶活性数据以及用抗II型精氨酸酶抗体进行的免疫沉淀,均证实这些克隆基因是啮齿动物的肝外型II型精氨酸酶。