Kim C S, Dorgan D R, Roe C R
Brain Res. 1984 Sep 17;310(1):149-53. doi: 10.1016/0006-8993(84)90019-2.
The organic anion transport system in the choroid plexus is responsible for excretion of organic anions from brain to plasma. Disruption of this system could then result in accumulation of encephalopathic acyl groups in brain in a variety of metabolic disorders. Octanoate produced inhibition of this transport system associated with disruption of mitochondrial ultrastructure. Octanoylcarnitine and L-carnitine had no effect. These compounds represent those seen in the medium chain acyl CoA dehydrogenase deficiency. L-Carnitine may be useful for protecting the central nervous system through formation of the non-toxic acylcarnitine in this and other metabolic disorders characterized by accumulation of encephalopathic metabolites.
脉络丛中的有机阴离子转运系统负责将有机阴离子从脑内排泄至血浆。该系统的破坏可能会导致在各种代谢紊乱中脑内出现脑病性酰基的蓄积。辛酸对该转运系统产生抑制作用，并伴有线粒体超微结构的破坏。辛酰肉碱和左旋肉碱则无此作用。这些化合物与中链酰基辅酶A脱氢酶缺乏症中所见的化合物相似。在这种以及其他以脑病性代谢物蓄积为特征的代谢紊乱中，左旋肉碱可能通过形成无毒的酰基肉碱来保护中枢神经系统。
