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过氧化物酶体、脂质代谢与过氧化物酶体疾病

Peroxisomes, lipid metabolism, and peroxisomal disorders.

作者信息

Wanders R J A

机构信息

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

出版信息

Mol Genet Metab. 2004 Sep-Oct;83(1-2):16-27. doi: 10.1016/j.ymgme.2004.08.016.

Abstract

Peroxisomes catalyse a large variety of different cellular functions of which most have to do with lipid metabolism. This paper deals with the role of peroxisomes in three key pathways of lipid metabolism, including: (1) etherphospholipid biosynthesis, (2) fatty acid beta-oxidation, and (3) fatty acid alpha-oxidation. Apart from a brief description of the peroxisomal enzymes involved in each of these pathways, the interaction between peroxisomes and other subcellular organelles, notably microsomes and peroxisomes, will be discussed. Finally, the current state of knowledge with respect to the different disorders of peroxisomal lipid metabolism will be described.

摘要

过氧化物酶体催化多种不同的细胞功能,其中大多数与脂质代谢有关。本文探讨过氧化物酶体在脂质代谢的三个关键途径中的作用,包括:(1)醚磷脂生物合成,(2)脂肪酸β-氧化,以及(3)脂肪酸α-氧化。除了简要描述参与这些途径的过氧化物酶体酶外,还将讨论过氧化物酶体与其他亚细胞器(特别是微粒体和过氧化物酶体)之间的相互作用。最后,将描述关于过氧化物酶体脂质代谢不同紊乱的当前知识状态。

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