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Metabolic interactions between peroxisomes and mitochondria with a special focus on acylcarnitine metabolism.
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease ( IF 4.2 ) Pub Date : 2020-02-10 , DOI: 10.1016/j.bbadis.2020.165720
Sander M Houten 1 , Ronald J A Wanders 2 , Pablo Ranea-Robles 1
Affiliation  

Carnitine plays an essential role in mitochondrial fatty acid β-oxidation as a part of a cycle that transfers long-chain fatty acids across the mitochondrial membrane and involves two carnitine palmitoyltransferases (CPT1 and CPT2). Two distinct carnitine acyltransferases, carnitine octanoyltransferase (COT) and carnitine acetyltransferase (CAT), are peroxisomal enzymes, which indicates that carnitine is not only important for mitochondrial, but also for peroxisomal metabolism. It has been demonstrated that after peroxisomal metabolism, specific intermediates can be exported as acylcarnitines for subsequent and final mitochondrial metabolism. There is also evidence that peroxisomes are able to degrade fatty acids that are typically handled by mitochondria possibly after transport as acylcarnitines. Here we review the biochemistry and physiological functions of metabolite exchange between peroxisomes and mitochondria with a special focus on acylcarnitines.

中文翻译:

过氧化物酶体与线粒体之间的代谢相互作用特别关注酰基肉碱的代谢。

肉碱在线粒体脂肪酸β-氧化中起重要作用,是循环长链脂肪酸跨线粒体膜转移并涉及两个肉碱棕榈酰转移酶(CPT1和CPT2)的循环的一部分。两种不同的肉碱酰基转移酶,肉碱辛酰基转移酶(COT)和肉碱乙酰基转移酶(CAT),是过氧化物酶体酶,这表明肉碱不仅对线粒体重要,对于过氧化物酶体代谢也很重要。已经证明过氧化物酶体代谢后,特定的中间体可以作为酰基肉碱输出,用于随后和最终的线粒体代谢。也有证据表明,过氧化物酶体能够降解通常由线粒体处理的脂肪酸,可能以酰基肉碱形式运输。
更新日期:2020-02-10
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