Costell M, Míguez M P, O'Connor J E, Grisolía S
Neurology. 1987 May;37(5):804-8. doi: 10.1212/wnl.37.5.804.
Decreased carnitine levels have been noted in conditions of hyperammonemia. We have measured carnitine and its derivatives in acute and sustained hyperammonemia in mice and studied the effect of carnitine administration thereon. Sustained hyperammonemia decreased carnitine in liver and muscle. Acetylcarnitine was decreased in liver and muscle in both acute and sustained hyperammonemia but increased in brain. Long-chain acylcarnitines decreased in brain and muscle in acute hyperammonemia and in liver and muscle is sustained ammonia intoxication. Intraperitoneal administration of carnitine increased the levels of free carnitine and acyl derivatives, especially in liver, but sustained hyperammonemia significantly affected the distribution of exogenous carnitine. The importance of these findings relative to the alterations of lipid metabolism observed in Reye's syndrome and inherited hyperammonemias, as well their implication in the protective effect of carnitine on hyperammonemia, are discussed.
在高氨血症状态下已观察到肉碱水平降低。我们测定了小鼠急性和持续性高氨血症时的肉碱及其衍生物，并研究了给予肉碱对其的影响。持续性高氨血症降低了肝脏和肌肉中的肉碱水平。在急性和持续性高氨血症时，肝脏和肌肉中的乙酰肉碱均降低，但在脑中升高。急性高氨血症时脑和肌肉中的长链酰基肉碱降低，而在持续性氨中毒时肝脏和肌肉中的长链酰基肉碱降低。腹腔注射肉碱可提高游离肉碱和酰基衍生物的水平，尤其是在肝脏中，但持续性高氨血症显著影响外源性肉碱的分布。讨论了这些发现相对于在瑞氏综合征和遗传性高氨血症中观察到的脂质代谢改变的重要性，以及它们对肉碱对高氨血症保护作用的影响。
