Metabolic interactions between peroxisomes and mitochondria with a special focus on acylcarnitine metabolism

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

卷:1866

Review

Houten, Sander M.¹  Wanders, Ronald J. A.²  Ranea-Robles, Pablo¹ 

[1] Icahn Sch Med Mt Sinai, Icahn Inst Data Sci & Genom Technol, Dept Genet & Genom Sci, 1425 Madison Ave,Box 1498, New York, NY 10029 USA

[2] Univ Amsterdam, Dept Clin Chem, Amsterdam Gastroenterol & Metab, Amsterdam UMC,Lab Genet Metab Dis, Meibergdreef 9, NL-1105 AZ Amsterdam, Netherlands

关键词: Peroxisome;    Mitochondria;    Carnitine;    Metabolite transport;    Fatty acid beta-oxidation;   

DOI  :  10.1016/j.bbadis.2020.165720

来源: Elsevier

【 摘 要 】

Carnitine plays an essential role in mitochondrial fatty acid beta-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.

【 授权许可】

Free