Anti-HADHA Rabbit Monoclonal Antibody for WB, IF/ICC, ELISA - P40939

Core Product Specifications and Parameters

Biological Background: HADHA Function and Localization

• Trifunctional enzyme subunit alpha, mitochondrial (also known as 78 kDa gastrin-binding protein, TP-alpha) is encoded by the HADHA gene.
• HADHA is the alpha subunit of the mitochondrial trifunctional protein complex, which catalyzes the last three steps of the long-chain fatty acid beta-oxidation cycle: hydration, dehydrogenation, and thiolysis. Related references: PMID:1550553, PMID:29915090, PMID:30850536, PMID:8135828, PMID:8163672
• The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues; each cycle shortens fatty acyl-CoA by two carbons, yielding acetyl-CoA, NADH, and FADH2 for ATP production. Related references: PMID:29915090
• HADHA specifically bears the 2,3-enoyl-CoA hydratase and 3-hydroxyacyl-CoA dehydrogenase activities, while the beta subunit (HADHB) carries the 3-ketoacyl-CoA thiolase activity. Related references: PMID:29915090, PMID:30850536, PMID:8135828
• The enzyme shows specificity for long-chain fatty acids, including saturated species such as palmitate and laurate, as well as unsaturated fatty acids like oleate, linoleate, and linolenate. Related references: PMID:1550553, PMID:8135828, PMID:8163672, PMID:26474213
• Independently of beta-oxidation, HADHA exhibits monolysocardiolipin acyltransferase activity to remodel cardiolipin, a critical mitochondrial membrane phospholipid. Related references: PMID:23152787, PMID:31604922
• Subcellular localization: Mitochondrion inner membrane.
• HADHA is associated with diseases; mutations in this gene cause mitochondrial trifunctional protein deficiency and related disorders.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 545–763 of human HADHA; this C-terminal region may detect full-length and certain truncated isoforms.
• For Western blot, a band around 83 kDa is expected; consider using mitochondrial-rich tissue lysates (e.g., liver, heart) for optimal signal.
• Cross-reactivity with mouse and rat is reported; validate in the relevant sample system before use.
• In IF/ICC, a mitochondrial staining pattern is anticipated; include appropriate organelle markers for verification.
• For ELISA applications, pair with a suitable detection antibody and optimize assay conditions.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio offers a comprehensive range of IVD raw materials including validated antibody pairs, monoclonal antibodies like this anti-HADHA mAb, and bulk ancillary reagents to support diagnostic manufacturers in fatty acid metabolism and mitochondrial disease research. As your sourcing bridge from concept to clinic, we also specialize in rare target raw-material procurement to meet your development needs.