过氧化物酶体、脂质代谢与脂毒性

Peroxisomes, lipid metabolism and lipotoxicity.

作者信息

Wanders R J A, Ferdinandusse S, Brites P, Kemp S

机构信息

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

出版信息

Biochim Biophys Acta. 2010 Mar;1801(3):272-80. doi: 10.1016/j.bbalip.2010.01.001. Epub 2010 Jan 12.

DOI:10.1016/j.bbalip.2010.01.001

PMID:20064629

Abstract

Peroxisomes play an essential role in cellular lipid metabolism as exemplified by the existence of a number of genetic diseases in humans caused by the impaired function of one of the peroxisomal enzymes involved in lipid metabolism. Key pathways in which peroxisomes are involved include: (1.) fatty acid beta-oxidation; (2.) etherphospholipid biosynthesis, and (3.) fatty acid alpha-oxidation. In this paper we will describe these different pathways in some detail and will provide an overview of peroxisomal disorders of metabolism and in addition discuss the toxicity of the intermediates of peroxisomal metabolism as they accumulate in the different peroxisomal deficiencies.

摘要

过氧化物酶体在细胞脂质代谢中起着至关重要的作用，这一点可由人类中一些由参与脂质代谢的过氧化物酶体酶功能受损引起的遗传疾病得到例证。过氧化物酶体所涉及的关键途径包括：（1）脂肪酸β-氧化；（2）醚磷脂生物合成，以及（3）脂肪酸α-氧化。在本文中，我们将详细描述这些不同的途径，并概述过氧化物酶体代谢紊乱，此外还将讨论过氧化物酶体代谢中间体在不同过氧化物酶体缺陷中积累时的毒性。