Anti-HADHB Antibody

HADHB encodes the beta subunit of the mitochondrial trifunctional protein, a hetero-octameric enzyme complex anchored to the inner mitochondrial membrane that drives three consecutive steps in long-chain fatty acid β-oxidation: enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase activity. Loss-of-function variants in HADHB cause mitochondrial trifunctional protein deficiency, an inherited metabolic disorder presenting with hypoketotic hypoglycemia, cardiomyopathy, and skeletal myopathy, making reliable detection of this subunit valuable in metabolic disease research and mitochondrial biology studies.

This rabbit polyclonal reagent from Antibodies.com is raised against HADHB and supplied as an unconjugated preparation in a 100 µL volume, validated for Western blot and immunofluorescence workflows. It cross-reacts with human, mouse, and rat samples, supporting comparative studies across standard model organisms and patient-derived material. The polyclonal format enables recognition of multiple epitopes on the target, which can be useful when probing processed or partially degraded forms of the protein in cell lysates and tissue sections.

For Western blot, the antibody can be applied to mitochondrial-enriched fractions or whole-cell lysates to assess expression of the trifunctional protein beta subunit, while immunofluorescence applications allow visualization of mitochondrial morphology and distribution in cultured cells. Aliquoting upon first use and storage at -20°C in working volumes helps preserve activity across multiple experiments.