Eaton S, Bartlett K, Pourfarzam M
Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle-upon-Tyne, U.K.
Biochem J. 1996 Dec 1;320 ( Pt 2)(Pt 2):345-57. doi: 10.1042/bj3200345.
The enzymic stages of mammalian mitochondrial beta-oxidation were elucidated some 30-40 years ago. However, the discovery of a membrane-associated multifunctional enzyme of beta-oxidation, a membrane-associated acyl-CoA dehydrogenase and characterization of the carnitine palmitoyl transferase system at the protein and at the genetic level has demonstrated that the enzymes of the system itself are incompletely understood. Deficiencies of many of the enzymes have been recognized as important causes of disease. In addition, the study of these disorders has led to a greater understanding of the molecular mechanism of beta-oxidation and the import, processing and assembly of the beta-oxidation enzymes within the mitochondrion. The tissue-specific regulation, intramitochondrial control and supramolecular organization of the pathway is becoming better understood as sensitive analytical and molecular techniques are applied. This review aims to cover enzymological and organizational aspects of mitochondrial beta-oxidation together with the biochemical aspects of inherited disorders of beta-oxidation and the intrinsic control of beta-oxidation.
大约在30到40年前，哺乳动物线粒体β-氧化的酶促阶段就已阐明。然而，膜相关β-氧化多功能酶、膜相关酰基辅酶A脱氢酶的发现以及肉碱棕榈酰转移酶系统在蛋白质和基因水平上的表征表明，该系统本身的酶尚未被完全了解。许多酶的缺乏已被确认为疾病的重要病因。此外，对这些疾病的研究加深了我们对β-氧化分子机制以及β-氧化酶在线粒体内的导入、加工和组装的理解。随着灵敏的分析和分子技术的应用，该途径的组织特异性调节、线粒体内控制和超分子组织正被更好地理解。本综述旨在涵盖线粒体β-氧化的酶学和组织学方面，以及β-氧化遗传性疾病的生化方面和β-氧化的内在控制。
