HADHB Antibody (C-6): sc-271496

HADHB Antibody (C-6) is a mouse monoclonal IgG1 antibody that detects HADHB in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). HADHB, or Trifunctional enzyme subunit beta (mitochondrial), is a crucial 474 amino acid protein encoded by the human gene HADHB, belonging to the thiolase family of enzymes. These enzymes play a vital role in fatty acid metabolism by catalyzing the reversible thiolytic cleavage of 3-ketoacyl-CoA into acyl-CoA and acetyl-CoA, a two-step reaction that involves a covalent intermediate formed with a catalytic cysteine. HADHB′s location in the mitochondrion is essential for the efficient processing of fatty acids, which are key energy sources for cellular functions. Defects in HADHB can lead to trifunctional protein deficiency, a condition characterized by a range of clinical manifestations including hypoglycemia, cardiomyopathy, and sudden death, highlighting HADHB′s critical role in metabolic health. HADHB forms an octameric structure composed of four alpha (HADHA) and four beta (HADHB) subunits, which is essential for enzymatic activity and stability. Understanding HADHB′s function and significance is crucial for developing therapeutic strategies for conditions associated with HADHB deficiency.

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HADHB Antibody (C-6) References:

1. HADHB, HuR, and CP1 bind to the distal 3'-untranslated region of human renin mRNA and differentially modulate renin expression.  |  Adams, DJ., et al. 2003. J Biol Chem. 278: 44894-903. PMID: 12933794
2. Isolated mitochondrial long-chain ketoacyl-CoA thiolase deficiency resulting from mutations in the HADHB gene.  |  Das, AM., et al. 2006. Clin Chem. 52: 530-4. PMID: 16423905
3. Estrogen receptor alpha interacts with mitochondrial protein HADHB and affects beta-oxidation activity.  |  Zhou, Z., et al. 2012. Mol Cell Proteomics. 11: M111.011056. PMID: 22375075
4. Estrogen receptor beta interacts and colocalizes with HADHB in mitochondria.  |  Zhou, Z., et al. 2012. Biochem Biophys Res Commun. 427: 305-8. PMID: 23000159
5. A compound heterozygous mutation in HADHB gene causes an axonal Charcot-Marie-tooth disease.  |  Hong, YB., et al. 2013. BMC Med Genet. 14: 125. PMID: 24314034
6. Mutations in HADHB, which encodes the β-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy.  |  Naiki, M., et al. 2014. Am J Med Genet A. 164A: 1180-7. PMID: 24664533
7. Posttranscriptional Regulation of 14q32 MicroRNAs by the CIRBP and HADHB during Vascular Regeneration after Ischemia.  |  Downie Ruiz Velasco, A., et al. 2019. Mol Ther Nucleic Acids. 14: 329-338. PMID: 30665182
8. Identification and functional characterization of mutations within HADHB associated with mitochondrial trifunctional protein deficiency.  |  Liu, ZR., et al. 2019. Mitochondrion. 49: 200-205. PMID: 31521624
9. Novel mutations in the HADHB gene causing a mild phenotype of mitochondrial trifunctional protein (MTP) deficiency.  |  Ørstavik, K., et al. 2022. JIMD Rep. 63: 193-198. PMID: 35433169
10. The Role of HADHB in Mitochondrial Fatty Acid Metabolism During Initiation of Metastasis in ccRCC.  |  Li, X., et al. 2025. Mol Carcinog.. PMID: 39991877

The preferred HADHB antibody is HADHB Antibody (E-1): sc-271495.