Rabbit anti-Human HADHB Polyclonal Antibody | anti-HADHB antibody

HADHB, CT (HADHB, Trifunctional enzyme subunit beta, mitochondrial, TP-beta, 3-ketoacyl-CoA thiolase, Acetyl-CoA acyltransferase, Beta-ketothiolase) (FITC)

Cat. Num. AAA6305331

• Gene Names: HADHB; ECHB; MTPB; MSTP029; TP-BETA
• Reactivity: Human
• Applications: Flow Cytometry, Functional Assay, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: HADHB; Polyclonal Antibody; CT (HADHB; Trifunctional enzyme subunit beta; mitochondrial; TP-beta; 3-ketoacyl-CoA thiolase; Acetyl-CoA acyltransferase; Beta-ketothiolase) (FITC); Beta-ketothiolase; anti-HADHB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-HADHB antibody: Flow Cytometry (FC/FACS), FLISA, Western Blot (WB)
• Application Notes: FC/FACS: 1:10-50; FLISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: HADHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 321~351 amino acids from the C-terminal region of human HADHB.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of HADHB Antibody (C-term) in Jurkat cell line lysates (35ug/lane). HADHB (arrow) was detected using the purified Pab.)

Flow Cytometry (FC/FACS)

(HADHB Antibody (C-term) flow cytometric analysis of Jurkat cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-HADHB antibody

HADHB encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.

Product Categories/Family for anti-HADHB antibody

Antibodies; Enzymes; Acyltransferase

References

Purevsuren, J., et al. Mol. Genet. Metab. 98(4):372-377(2009). Bogenhagen, D.F., et al. J. Biol. Chem. 283(6):3665-3675(2008). Wang, R., et al. Zhonghua Fu Chan Ke Za Zhi 41(10):672-675(2006). Hillier, L.W., et al. Nature 434(7034):724-731(2005).

NCBI and Uniprot Product Information

• NCBI GI #: 4504327
• NCBI GeneID: 3032
• NCBI Accession #: NP_000174.1
• NCBI GenBank Nucleotide #: NM_000183.2
• UniProt Accession #: P55084; O14969; Q53TA6; Q96C77; Q9H3F5; Q9T2V8; B2RB16; B4E2W0
• Molecular Weight: 48,880 Da
• NCBI Official Full Name: trifunctional enzyme subunit beta, mitochondrial isoform 1
• NCBI Official Synonym Full Names: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
• NCBI Official Symbol: HADHB
• NCBI Official Synonym Symbols: ECHB; MTPB; MSTP029; TP-BETA
• NCBI Protein Information: trifunctional enzyme subunit beta, mitochondrial
• UniProt Protein Name: Trifunctional enzyme subunit beta, mitochondrial
• Protein Family: Trifunctional enzyme
• UniProt Gene Name: HADHB
• UniProt Entry Name: ECHB_HUMAN

Uniprot Description

HADHB: Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency). The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex. Belongs to the thiolase family.

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.16; Lipid Metabolism - fatty acid; Lipid Metabolism - fatty acid elongation in mitochondria; Transferase

Chromosomal Location of Human Ortholog: 2p23

Cellular Component: endoplasmic reticulum; mitochondrial envelope; mitochondrial inner membrane; mitochondrial outer membrane

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; enoyl-CoA hydratase activity; protein binding

Biological Process: fatty acid beta-oxidation

Disease: Trifunctional Protein Deficiency