MitoSciences: Delta(3,5)-delta(2,4)-dienoyl-CoA isomerase (ECH1)

UniProt Number:	Q13011
Structure and Function:	ECH1 is a member of the hydratase/isomerase superfamily involved in fatty acid metabolism. It is localized to peroxisomes and/or mitochondria in different mammals. In humans, levels of ECH1 are modulated by peroxisome proliferators. As the name implies, ECH1 catalyzes the isomerization of cis Δ3,5, double bonds in unsaturated acyl-CoAs to yield trans Δ2,4-dienoyl CoAs. These are substrates for Δ2,4-dienoyl CoA reductase which produces trans Δ3 enoyl CoA. This intermediate can then be further transformed by Δ3, Δ2 - enoyl CoA isomerase to finally yield the trans-2 double bond configured fatty acyl CoA, which can be then directed into the β-oxidation pathway. Expression of ECH1 is highest in metabolically active tissues such as heart, skeletal muscle, central nervous system, liver and kidney. Recent studies show that it is down regulated in gastric carcinoma and CLL.
Disease Associations:	n/a