Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
Volume 1866, Issue 5, Pages -Publisher
ELSEVIER
DOI: 10.1016/j.bbadis.2020.165720
Keywords
Peroxisome; Mitochondria; Carnitine; Metabolite transport; Fatty acid beta-oxidation
Funding
- National Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health [R01DK113172]
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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.
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