Textbook biochemistry describes that most fatty acids are synthesized in the cytoplasm and contain a linear chain of carbon atoms. While this is correct, it does not represent the entire spectrum of fatty acid synthesis, since some fatty acids contain branches in their carbon backbone. Furthermore, an independent fatty acid synthesis system exists in mitochondria. We only know little about how these ‘unusual’ pathways contribute to physiology and disease. We want to change this!


Dewulf JP, Paquay S, Marbaix E, Achouri Y, Van Schaftingen E, Bommer GT (2021). ECHDC1 knockout mice accumulate ethyl-branched lipids and excrete abnormal intermediates of branched-chain fatty acid metabolism. J Biol Chem. 297(4):101083. doi: 10.1016/j.jbc.2021.101083.

Dewulf JP, Gerin I, Rider MH, Veiga-da-Cunha M, Van Schaftingen E, Bommer GT (2019). The synthesis of branched-chain fatty acids is limited by enzymatic decarboxylation of ethyl- and methylmalonyl-CoA. Biochemical Journal. 476(16):2427-2447. doi: 10.1042/BCJ20190500.