过氧化物酶体及其在人类代谢相互作用网络中的核心作用。

Peroxisomes and Their Central Role in Metabolic Interaction Networks in Humans.

作者信息

Wanders Ronald J A, Waterham Hans R, Ferdinandusse Sacha

机构信息

Laboratory Genetic Metabolic Diseases, Departments Clinical Chemistry and Pediatrics, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.

出版信息

Subcell Biochem. 2018;89:345-365. doi: 10.1007/978-981-13-2233-4_15.

DOI:10.1007/978-981-13-2233-4_15

PMID:30378031

Abstract

Peroxisomes catalyze a number of essential metabolic functions and impairments in any of these are usually associated with major clinical signs and symptoms. In contrast to mitochondria which are autonomous organelles that can catalyze the degradation of fatty acids, certain amino acids and other compounds all by themselves, peroxisomes are non-autonomous organelles which are highly dependent on the interaction with other organelles and compartments to fulfill their role in metabolism. This includes mitochondria, the endoplasmic reticulum, lysosomes, and the cytosol. In this paper we will discuss the central role of peroxisomes in different metabolic interaction networks in humans, including fatty acid oxidation, ether phospholipid biosynthesis, bile acid synthesis, fatty acid alpha-oxidation and glyoxylate metabolism.

摘要

过氧化物酶体催化多种重要的代谢功能，其中任何一种功能受损通常都与主要的临床体征和症状相关。与线粒体这种能够自行催化脂肪酸、某些氨基酸和其他化合物降解的自主细胞器不同，过氧化物酶体是非自主细胞器，高度依赖与其他细胞器和区室的相互作用来完成其在代谢中的作用。这包括线粒体、内质网、溶酶体和细胞质。在本文中，我们将讨论过氧化物酶体在人类不同代谢相互作用网络中的核心作用，包括脂肪酸氧化、醚磷脂生物合成、胆汁酸合成、脂肪酸α-氧化和乙醛酸代谢。