Anti-HADHA Antibody

From Antibodies.com, this unconjugated antibody was raised in rabbit against the human HADHA antigen for mitochondrial and metabolic research. HADHA encodes the alpha subunit of the mitochondrial trifunctional protein (MTP), an inner-membrane enzyme complex that catalyzes three sequential reactions of long-chain fatty acid β-oxidation: the enoyl-CoA hydratase, L-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase steps. Loss-of-function variants in HADHA underlie long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and broader MTP deficiency, two inherited fatty-acid-oxidation disorders whose molecular characterization has relied on antibody-based detection of the alpha subunit in patient fibroblasts and tissue biopsies.

The reagent has been validated across human, mouse, and rat samples in Western blot, immunohistochemistry, and immunofluorescence workflows, giving laboratories a single reagent line that covers the species typically used in fatty-acid-oxidation disease models. It is buffered in PBS with 0.1% sodium azide and 50% glycerol, supplied at approximately 1 mg/ml as a 100 µl liquid preparation. For best results, store the vial frozen at -20°C in single-use aliquots, limit freeze-thaw cycles, and protect from light during extended incubations.