HADHB antibody (ab10093)

Product Name 

HADHB antibody

Product type 

Primary antibodies

Description 

Goat polyclonal to HADHB

Immunogen 

Synthetic peptide: TILTYPFKNLPT, corresponding to N terminal amino acids 2-13 of Human HADHB.
BLAST 'TILTYPFKNLPT-C' with or

Reacts with 
(species key)

Hu

Tested applications 
(see key)

WB

Abreviews 

Customer reviews (see key)

Read about Abreviews (Read all Abreviews)

Application notes 
(see key)

Recommended dilutions
WB: Use at an assay dependent concentration.


Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.

Positive control 
(see definition)

Human brain and Jurkat extracts.

Cellular localization 

Mitochondrial

Research areas 

Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Metabolism of lipids and lipoproteins
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Mitochondrial

Relevance 

The HADHB gene 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. Alternatively spliced transcript variants have been found; however, their full-length nature is not known.

The HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy.
Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.

Database links 

Raised in 

Goat

Clonality 

Polyclonal

Isotype 

IgG

Purity 

Immunogen affinity purified

Storage buffer 

Preservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris-saline. pH 7.3

Material safety datasheet (MSDS) for this product:
Sodium Azide MSDS

Purification notes 

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Form 

Liquid

Concentration 

0.500 mg/ml

Storage instructions 

Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

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