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Abstract：
Peroxisomes are intracellular organelles that perform vital metabolic functions. They have been extensively studied in the hepatic and renal systems, yet their pivotal roles in facilitating central nervous system patterning and in disease pathogenesis are only recently being firmly established by the neuroscience community. Peroxisomal functions including the break-down of long chain fatty acids, the removal of H2O2, and the biosynthesis of ether lipids. The build up of long chain fatty acids and H2O2 is detrimental to cellular function, and ether lipids play roles in maintaining cell membrane structure. These findings have major implications for treatments for the full spectrum of peroxisomal disorders. Here, we provide a timely review highlighting the most important data in recent times linking peroxisomal functions to brain formation, and we describe how peroxisomal deficiency and pathway dysfunction results in neurological deficits, the more severe of which result in life changing disabilities and death.
摘要：
过氧化物酶体是执行重要代谢功能的细胞内细胞器。它们在肝脏和肾脏系统中得到了广泛的研究，然而，它们在促进中枢神经系统模式和疾病发病机制方面的关键作用，直到最近才被神经科学界牢固地确立。过氧化物酶体功能，包括长链脂肪酸的分解，H2O2的去除，以及醚类脂的生物合成。长链脂肪酸和H2O2的积累对细胞功能有害，醚类脂在维持细胞膜结构中发挥作用。这些发现对过氧化物酶体紊乱的全谱治疗具有重要意义。这里,我们提供了一个及时的审查，强调了最近将过氧化物酶体功能与大脑形成联系起来的最重要的数据，我们描述了过氧化物酶体缺乏和通路功能障碍如何导致神经功能缺损，其中更严重的导致改变生活的残疾和死亡。
