HADHB Antibody (D-10): sc-365907

HADHB Antibody (D-10) is a mouse monoclonal IgG1 antibody that detects HADHB in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), 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 efficient processing of fatty acids, which are key energy sources for cellular functions. HADHB exists as an octamer composed of four alpha (HADHA) and four beta (HADHB) subunits, and any defects in this complex can lead to trifunctional protein deficiency (TFP deficiency), a condition characterized by hypoglycemia, cardiomyopathy, and sudden death. The biochemical hallmark of TFP deficiency is the loss of all three enzyme activities of the trifunctional protein complex, highlighting HADHB′s critical role in maintaining metabolic homeostasis.

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HADHB Antibody (D-10) References:

1. Identification of novel mutations of the HADHA and HADHB genes in patients with mitochondrial trifunctional protein deficiency.  |  Choi, JH., et al. 2007. Int J Mol Med. 19: 81-7. PMID: 17143551
2. Comprehensive cDNA study and quantitative analysis of mutant HADHA and HADHB transcripts in a French cohort of 52 patients with mitochondrial trifunctional protein deficiency.  |  Boutron, A., et al. 2011. Mol Genet Metab. 103: 341-8. PMID: 21549624
3. A patient with mitochondrial trifunctional protein deficiency due to the mutations in the HADHB gene showed recurrent myalgia since early childhood and was diagnosed in adolescence.  |  Yagi, M., et al. 2011. Mol Genet Metab. 104: 556-9. PMID: 22000755
4. Noncompaction in mitochondrial trifunctional protein deficiency due to a HADHB mutation.  |  Finsterer, J. and Zarrouk-Majoub, S. 2015. Eur J Pediatr. 174: 1693-4. PMID: 26206388
5. Integrated analyses of multi-omics reveal global patterns of methylation and hydroxymethylation and screen the tumor suppressive roles of HADHB in colorectal cancer.  |  Zhu, Y., et al. 2018. Clin Epigenetics. 10: 30. PMID: 29507648
6. Trifunctional Protein Deficiency Due to HADHB Mutations Is a Multisystem, β-Oxidation Disorder.  |  Finsterer, J. and Zarrouk-Mahjoub, S. 2017. Arch Iran Med. 20: 767-769. PMID: 29664318
7. MTP deficiency caused by HADHB mutations: Pathophysiology and clinical manifestations.  |  Dagher, R., et al. 2021. Mol Genet Metab. 133: 1-7. PMID: 33744096
8. HADHB, a fatty acid beta-oxidation enzyme, is a potential prognostic predictor in malignant lymphoma.  |  Sekine, Y., et al. 2022. Pathology. 54: 286-293. PMID: 34531036
9. DNA demethylase TET2-mediated reduction of HADHB expression contributes to cadmium-induced malignant progression of colorectal cancer.  |  Li, L., et al. 2024. Ecotoxicol Environ Saf. 280: 116579. PMID: 38865940
10. Fluorescence in situ hybridization mapping of the alpha and beta subunits (HADHA and HADHB) of human mitochondrial fatty acid beta-oxidation multienzyme complex to 2p23 and their evolution.  |  Aoyama, T., et al. 1997. Cytogenet Cell Genet. 79: 221-4. PMID: 9605857

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