[KO Validated] Anti-HADHA Rabbit PolymAb® for WB, IHC-P, IF/ICC, IP, ELISA - P40939

Core Product Specifications and Parameters

Biological Background: HADHA Function and Localization

• Catalyzes the last three steps of mitochondrial beta-oxidation: 2,3-enoyl-CoA hydratase and 3-hydroxyacyl-CoA dehydrogenase, converting long-chain fatty acyl-CoAs to acetyl-CoA, generating reducing equivalents for ATP production (PubMed:1550553, PubMed:29915090, PubMed:30850536, PubMed:8135828, PubMed:8163672). Related references: PMID:1550553, PMID:29915090, PMID:30850536, PMID:8135828, PMID:8163672
• Forms a heterotetrameric complex with HADHB (subunit beta) that completes the beta-oxidation cycle with 3-ketoacyl-CoA thiolase activity (PubMed:29915090, PubMed:30850536, PubMed:8135828). Related references: PMID:29915090, PMID:30850536, PMID:8135828
• Shows substrate specificity for long-chain fatty acids such as palmitate, laurate, oleate, linoleate, and linolenate, derived from dietary and stored fats (PubMed:26474213). Related references: PMID:26474213
• Additionally possesses monolysocardiolipin acyltransferase activity, contributing to mitochondrial cardiolipin remodeling crucial for membrane architecture (PubMed:23152787, PubMed:31604922). Related references: PMID:23152787, PMID:31604922
• Located at the mitochondrial inner membrane; imported via an N-terminal transit peptide.
• Subject to multiple post-translational modifications including acetylation, phosphorylation, and methylation, which may regulate enzyme activity and stability.
• Genetic defects in HADHA cause mitochondrial trifunctional protein deficiency (MTPD), a severe inborn error of fatty acid metabolism.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the C-terminal region (aa 545-763) of human HADHA, ensuring specific recognition of the full-length protein. For Western blot, a major band at approximately 83 kDa is expected; optimize loading controls for your sample type.
• In immunohistochemistry (IHC-P), use appropriate antigen retrieval methods (heat-induced or enzymatic) and validate in positive control tissues expressing HADHA.
• For immunofluorescent staining (IF/ICC), fixation with methanol or paraformaldehyde followed by permeabilization is recommended due to mitochondrial localization; confirm mitochondrial co-localization with organelle markers.
• For immunoprecipitation and ELISA, pair with a detection antibody targeting a distinct epitope and validate assay conditions in your matrix.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio offers comprehensive raw-material solutions for research and diagnostic development in mitochondrial biology and metabolic disorders. With this anti-HADHA antibody, we provide validated monoclonal and polyclonal antibodies, antibody pairs, and bulk ancillary reagents to streamline your assay development. Our platform also supports custom sourcing for rare targets, ensuring you have a reliable partner from concept to clinic.