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Products:Signal Transduction >> Metabolism >> Mitochondrial
Overview
Product name
Anti-HADHB antibody
See all HADHB products (5) ...
Description
Goat polyclonal to HADHB
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Synthetic peptide: TILTYPFKNLPT, corresponding to N terminal amino acids 2-13 of Human HADHB.
TILTYPFKNL PT-C
Positive control
Human brain and Jurkat extracts.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris-saline. pH 7.3
Concentration
Concentration information loading...
Purity
Immunogen affinity purified
Purification notes
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Primary antibody notes
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.
Clonality
Polyclonal
Isotype
IgG
Applications
Show applications key
Our Abpromise guarantee covers the use of ab10093 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: Use at an assay dependent concentration.
Target
Pathway
Lipid metabolism; fatty acid beta-oxidation.
Involvement in disease
Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. 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.
Sequence similarities
Belongs to the thiolase family.
Cellular localization
Mitochondrion.
Target information above from: UniProt accessionP55084
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- 2 enoyl Coenzyme A (CoA) hydratase beta subunit antibody
- 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit antibody
- 3 ketoacyl Coenzyme A thiolase antibody
see all
Database links
- Entrez Gene: 3032 Human
- Omim: 143450 Human
- SwissProt: P55084 Human
- Unigene: 515848 Human
References for Anti-HADHB antibody (ab10093)
ab10093 has not yet been referenced specifically in any publications.
Publishing research using ab10093? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"