Alexander J J, Snyder A, Tonsgard J H
University of Chicago Pritzker School of Medicine, Department of Pediatrics, The Joseph P. Kennedy Mental Retardation Center, IL 60637, USA.
Neurochem Res. 1998 Feb;23(2):227-33. doi: 10.1023/a:1022441211177.
The accumulation of dicarboxylic acids is a prominent feature of inborn and toxin induced disorders of fatty acid metabolism which are characterized by impaired mental status. The formation of dicarboxylic acids is also a critical step in liver in the induction of intracellular fatty acid binding proteins and the proliferation of peroxisomes. In order to understand what potential roles dicarboxylic acids have in brain, we examined the extent of omega-oxidation in rat brain. Homogenates of rat brain catalyze the omega-oxidation of monocarboxylic acids with a specific activity of between 0.87 and 5.23 nmol/mg of post-mitochondrial protein/h, depending on the substrate. The activity is remarkably high, between one-fourth and 4 times the activity found in rat liver, depending on the chain length of the substrate. Specific activity increases with increasing chain length of the substrate. The omega-oxidation of palmitic acid is linear over a range of 0.125-3.0 mg of protein and 5-50 microM substrate for up to 45 minutes of incubation. The product of omega-oxidation in brain is almost exclusively dicarboxylic acid. Cultured rat neurons, astrocytes, and oligodendrocytes all contain omega-oxidation activity. Western blots of rat brain homogenate demonstrate a protein that is recognized by antibody to rat liver CYP4A omega-hydroxylase. These results demonstrate that the omega-oxidative pathway is prominent in brain and could play a role in brain fatty acid metabolism.
二羧酸的积累是先天性和毒素诱导的脂肪酸代谢紊乱的一个突出特征,这些紊乱以精神状态受损为特征。二羧酸的形成也是肝脏中诱导细胞内脂肪酸结合蛋白和过氧化物酶体增殖的关键步骤。为了了解二羧酸在大脑中可能发挥的作用,我们检测了大鼠大脑中ω-氧化的程度。大鼠大脑匀浆催化单羧酸的ω-氧化,其比活性在0.87至5.23 nmol/毫克线粒体后蛋白/小时之间,具体取决于底物。该活性非常高,根据底物的链长,是大鼠肝脏中活性的四分之一到4倍。比活性随着底物链长的增加而增加。在0.125 - 3.0毫克蛋白质和5 - 50微摩尔底物的范围内,棕榈酸的ω-氧化在长达45分钟的孵育过程中呈线性。大脑中ω-氧化的产物几乎完全是二羧酸。培养的大鼠神经元、星形胶质细胞和少突胶质细胞都含有ω-氧化活性。大鼠大脑匀浆的蛋白质免疫印迹显示有一种蛋白质能被抗大鼠肝脏CYP4A ω-羟化酶的抗体识别。这些结果表明,ω-氧化途径在大脑中很突出,可能在大脑脂肪酸代谢中发挥作用。