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Humans and other animals burn fat for energy in a process called fatty acid oxidation. The machinery for fatty acid oxidation is present in two subcellular compartments or organelles. Mitochondria, commonly referred to as the powerhouse of the cell, burn most of the fats. A second organelle named the peroxisome oxidizes a smaller amount of specific fatty acids, but can also handle fats that are normally oxidized within mitochondria. In this work, the investigators studied the crosstalk between mitochondria and peroxisomes. They investigated the mechanism by which peroxisomes handle fatty acids that accumulate when mitochondrial oxidation is defective or overloaded. This is a relevant process because of the existence of genetic diseases in which mitochondrial fatty acid oxidation is defective. Patients with such a disorder may suffer from low blood sugar during fasting, and heart and skeletal muscle disease. The investigators used genetic modification in cell lines and mice to manipulate mitochondrial and peroxisomal fatty acid oxidation and observed the biochemical consequences. This led to the identification of specific players that are crucial in the crosstalk between these two organelles. More work is necessary to study the role of this pathway in the disease processes of mitochondrial fatty acid oxidation disorders, and to evaluate potential therapeutic applications.
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This page is a summary of: Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4, The FASEB Journal, December 2018, Federation of American Societies For Experimental Biology (FASEB),
DOI: 10.1096/fj.201801498r.
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