Fatty Acid Metabolism in Peroxisomes and Related Disorders.

4区医学 Q2 Biochemistry, Genetics and Molecular Biology Advances in experimental medicine and biology Pub Date : 2024-01-01 DOI:10.1007/5584_2024_802

Katsuya Morito, Hanif Ali, Shigenobu Kishino, Tamotsu Tanaka

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Abstract

One of the functions of peroxisomes is the oxidation of fatty acids (FAs). The importance of this function in our lives is evidenced by the presence of peroxisomal disorders caused by the genetic deletion of proteins involved in these processes. Unlike mitochondrial oxidation, peroxisomal oxidation is not directly linked to ATP production. What is the role of FA oxidation in peroxisomes? Recent studies have revealed that peroxisomes supply the building blocks for lipid synthesis in the endoplasmic reticulum and facilitate intracellular carbon recycling for membrane quality control. Accumulation of very long-chain fatty acids (VLCFAs), which are peroxisomal substrates, is a diagnostic marker in many types of peroxisomal disorders. However, the relationship between VLCFA accumulation and various symptoms of these disorders remains unclear. Recently, we developed a method for solubilizing VLCFAs in aqueous media and found that VLCFA toxicity could be mitigated by oleic acid replenishment. In this chapter, we present the physiological role of peroxisomal FA oxidation and the knowledge obtained from VLCFA-accumulating peroxisome-deficient cells.

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过氧化物酶体中的脂肪酸代谢及相关疾病。

过氧物酶体的功能之一是氧化脂肪酸（FA）。这一功能在我们生活中的重要性体现在过氧化物酶体疾病的存在上，这种疾病是由于基因中缺失了参与这些过程的蛋白质而引起的。与线粒体氧化不同，过氧物酶体氧化与 ATP 的产生没有直接联系。FA 氧化在过氧物酶体中的作用是什么？最近的研究发现，过氧物酶体为内质网的脂质合成提供了构件，并促进了细胞内碳的循环以控制膜的质量。作为过氧物酶体底物的超长链脂肪酸（VLCFAs）的积累是多种过氧物酶体疾病的诊断标志。然而，VLCFA 的积累与这些疾病的各种症状之间的关系仍不清楚。最近，我们开发了一种在水介质中溶解 VLCFA 的方法，并发现补充油酸可以减轻 VLCFA 的毒性。在本章中，我们将介绍过氧物酶体FA氧化的生理作用以及从VLCFA富集的过氧物酶体缺陷细胞中获得的知识。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Advances in experimental medicine and biology 医学-医学：研究与实验

CiteScore

5.90

自引率

0.00%

发文量

465

审稿时长

2-4 weeks

期刊介绍： Advances in Experimental Medicine and Biology provides a platform for scientific contributions in the main disciplines of the biomedicine and the life sciences. This series publishes thematic volumes on contemporary research in the areas of microbiology, immunology, neurosciences, biochemistry, biomedical engineering, genetics, physiology, and cancer research. Covering emerging topics and techniques in basic and clinical science, it brings together clinicians and researchers from various fields.

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