Anti-ECHS1 Antibody

Raised in rabbit against a recombinant fusion protein covering amino acids 28–290 of human ECHS1 (NP_004083.3), this polyclonal IgG reagent from Antibodies.com binds the mitochondrial enzyme Enoyl-CoA Hydratase Short Chain 1, which catalyzes the hydration of trans-2-enoyl-CoA intermediates during fatty acid beta-oxidation. The unconjugated antiserum is supplied as 200 µl of affinity-purified polyclonal antibody in PBS (pH 7.3) containing 0.02% sodium azide and 50% glycerol, and it is shipped at 4°C for laboratory use; upon arrival it should be aliquoted and stored at -20°C to minimize freeze-thaw cycles.

Validated for Western blot and immunofluorescence, the antibody detects the expected ~31 kDa ECHS1 band on denaturing immunoblots and produces characteristic mitochondrial staining in cultured cells, with recommended dilutions of 1:1000–1:2000 for WB and 1:50–1:200 for IF. Cross-reactivity with human, mouse, and rat samples enables comparative studies of mitochondrial matrix proteins in models of short-chain enoyl-CoA hydratase deficiency, Leigh syndrome, and altered valine catabolism. Because the reagent is unconjugated, it can be paired with HRP-, fluorophore-, or biotin-conjugated secondary antibodies for flexible detection strategies, supporting research use only workflows in mitochondrial metabolism and inborn errors of fatty acid oxidation.