Anti-ECHS1 Antibody

The ECHS1 gene encodes enoyl-CoA hydratase, short chain 1, a mitochondrial matrix enzyme that catalyzes the hydration of trans-2-enoyl-CoA intermediates during fatty acid beta-oxidation. Antibodies.com offers a rabbit polyclonal antibody raised against this target, validated for Western blot and immunofluorescence workflows and reactive with human, mouse, and rat samples.

ECHS1 variants cause mitochondrial short-chain enoyl-CoA hydratase deficiency, an inherited metabolic condition presenting with lactic acidosis and Leigh-like encephalopathy, so a well-characterized antibody against the protein supports research into mitochondrial fatty acid oxidation, metabolic phenotyping, and disease mechanism studies. The reagent is supplied as 50 µl of unconjugated rabbit IgG in a single-use vial, with polyclonal clonality confirmed, allowing flexible secondary conjugation or direct detection in reducing Western blot gels and fixed-cell immunofluorescence imaging.