瞬态hyperammonemia由于尿素循环酶缺乏症在爱尔兰猎狼犬。

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Zandvliet MM, Rothuizen J

瞬态hyperammonemia由于尿素循环酶缺乏症在爱尔兰猎狼犬。

J兽医实习生地中海。2007;3 - 4月21 (2):215 - 8。

PubMed ID

17427379 (在PubMed

]

文摘

背景:爱尔兰猎狼犬经常有先天性门体静脉的分流,但相当比例的6 - 8-wk-old幼崽已经hyperammonemia没有门体静脉的分流。这hyperammonemia造成没有信号和瞬变,3 - 4个月岁正常化。假设:瞬态hyperammonemia在爱尔兰猎狼犬代谢的基础上。动物:两个相关(相同陛下)窝的爱尔兰猎狼犬(17幼崽)和他们的父母进行了研究。方法:门脉循环的完整性检查和超声显像。没有实质的肝脏疾病被肝脏活检证实。氨基酸概要文件在4幼崽和重复测定2这些小狗当氨浓度的规范化。氨基酸的资料比较与健康的爱尔兰猎狼犬幼崽。结果:空腹静脉氨浓度高(113 - 622年microg / dL 65 - 345 micromol / L)在所有的幼崽,而胆汁酸在参考范围内除了1。氨和胆汁酸浓度从所有的父母在参考值范围内。 Portosystemic shunting was excluded in all but 1 pup. Liver biopsy excluded significant lesions in all 10 pups examined. Hypercitrullinemia was found and persisted even when ammonia had normalized, at the expense of an increase in glutamine and asparagine. CONCLUSIONS AND CLINICAL IMPORTANCE: Citrulline concentrations are controlled by the urea cycle enzymes argininosuccinase and argininosuccinate synthetase, and a defect in either of these enzymes may be responsible for the transient hyperammonemia in Irish Wolfhounds. Resolution of the hyperammonemia is associated with increased activity of alternative metabolic pathways forming glutamine and asparagine. Confirmation requires measurement of enzyme activities in liver tissue.

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药物靶点

药物	目标	类	生物	药理作用	行动
瓜氨酸	Argininosuccinate合成酶,同种型CRA_a	蛋白质	人类	未知的	不可用	细节