Description_x000D_ Immunogen_x000D_ ECHS1 (AAH08906, 13 a.a. ~ 290 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa._x000D_ Sequence_x000D_ GPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ_x000D_ Physical form_x000D_ Solution_x000D_ Legal Information_x000D_ GenBank is a registered trademark of United States Department of Health and Human Services_x000D_ Biochem/physiol Actions_x000D_ ECHS1 (Enoyl CoA hydratase, short chain 1, mitochondrial) performs in the mitochondrial β-oxidation of fatty acid by catalyzing the second step of the process i.e. hydration of 2-trans-enoyl-coenzyme A (CoA) to L-3-hydroxyacyl-CoAs. It also participates in different metabolic pathways involving fatty acids and amino acids, valine. Study reports that heterozygous mutation in ECHS1 causes Leigh syndrome with hypotonia, metabolic acidosis, and developmental delay symptoms.