School of Life Sciences, Kyungpook National University, 80 Daehakro Bukgu, Daegu, 41566, Republic of Korea.
Arch Pharm Res. 2019 May;42(5):393-406. doi: 10.1007/s12272-019-01131-2. Epub 2019 Feb 9.
Peroxisomes and their (patho-)physiological importance in heath and disease have attracted increasing interest during last few decades. Together with mitochondria, peroxisomes comprise key metabolic platforms for oxidation of various fatty acids and redox regulation. In addition, peroxisomes contribute to bile acid, cholesterol, and plasmalogen biosynthesis. The importance of functional peroxisomes for cellular metabolism is demonstrated by the marked brain and systemic organ abnormalities occuring in peroxisome biogenesis disorders and peroxisomal enzyme deficiencies. Current evidences indicate that peroxisomal function is declined with aging, with peroxisomal dysfunction being linked to early onset of multiple age-related diseases including neurodegenerative diseases. Herein, we review recent progress toward understanding the physiological roles and pathological implications of peroxisomal dysfunctions, focusing on neurodegenerative disease.
几十年来，过氧化物酶体及其（病理）生理学在健康和疾病中的重要性引起了越来越多的关注。过氧化物酶体与线粒体一起，是各种脂肪酸氧化和氧化还原调节的关键代谢平台。此外，过氧化物酶体有助于胆汁酸、胆固醇和磷脂的生物合成。过氧化物酶体生物发生障碍和过氧化物酶体酶缺乏症中明显的脑和全身器官异常表明，功能性过氧化物酶体对细胞代谢很重要。目前的证据表明，过氧化物体的功能随年龄的增长而下降，过氧化物体功能障碍与多种与年龄相关的疾病（包括神经退行性疾病）的早期发病有关。本文综述了近年来对过氧化物酶体功能障碍的生理作用和病理意义的理解进展，重点介绍了神经退行性疾病。
