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过氧化物酶体β-氧化的病理生理学

Pathophysiology of peroxisomal beta-oxidation.

作者信息

Vamecq J, Draye J P

机构信息

Laboratoire de Chimie Physiologique, International Institute of Cellular and Molecular Pathology, Brussels, Belgium.

出版信息

Essays Biochem. 1989;24:115-225.

PMID:2676523
Abstract

Mammalian peroxisomes are subcellular organelles involved in the metabolism of hydrogen peroxide (oxidases, catalase), lipid anabolism (ether lipid biosynthesis) and catabolism (oxidation of fatty acids and fatty acid derivatives), and intermediary metabolism (transaminases, dehydrogenases). Peroxisomes are formed by division, as is the case for mitochondria, but, in contrast to these organelles, they do not contain DNA. They were discovered and characterized (by biochemical and morphological techniques) later than the majority of the other cell components and specific procedures have been developed for their isolation. Functions of peroxisomes are, as a rule, shared by other cell compartments so that specific enzyme assays have also been developed. Combination of specific isolation procedures, enzyme assays and morphological analysis have resulted in our current knowledge of peroxisomal physiology which, however, has greatly benefited, as in the case of lysosomes, from the study of inborn errors of metabolism and the contribution of molecular biology. Novel enzymes and metabolic pathways have been demonstrated to exist in peroxisomes and human genetic disorders affecting one or several of these functions have been recognized.

摘要

哺乳动物过氧化物酶体是一种亚细胞细胞器,参与过氧化氢代谢(氧化酶、过氧化氢酶)、脂质合成代谢(醚脂生物合成)和分解代谢(脂肪酸及脂肪酸衍生物氧化)以及中间代谢(转氨酶、脱氢酶)。过氧化物酶体通过分裂形成,线粒体也是如此,但与这些细胞器不同的是,过氧化物酶体不含DNA。它们是在大多数其他细胞成分之后被发现并通过生化和形态学技术进行表征的,并且已经开发出了用于分离它们的特定程序。过氧化物酶体的功能通常由其他细胞区室共同承担,因此也开发了特定的酶测定方法。特定的分离程序、酶测定和形态分析相结合,形成了我们目前对过氧化物酶体生理学的认识,然而,与溶酶体的情况一样,过氧化物酶体生理学在很大程度上受益于对先天性代谢缺陷的研究以及分子生物学的贡献。已证明过氧化物酶体中存在新的酶和代谢途径,并且已经识别出影响其中一项或多项功能的人类遗传疾病。

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1
Pathophysiology of peroxisomal beta-oxidation.过氧化物酶体β-氧化的病理生理学
Essays Biochem. 1989;24:115-225.
2
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