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Products:Signal Transduction >> Metabolism >> Mitochondrial
Overview
Product name
Anti-HADHB antibody
See all HADHB products (5) ...
Description
Mouse polyclonal to HADHB
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Recombinant full length Human HADHB protein (NP_000174.1).
Positive control
Human liver lysate, A431 cell lysate, and HADHB transfected 293T cell lysate.
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: None
Constituents: 1X PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein A purified
Clonality
Polyclonal
Isotype
IgG
Applications
Show applications key
Our Abpromise guarantee covers the use of ab88256 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: Use a concentration of 1 µg/mlPredicted molecular weight: 51 kDa.
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
Anti-HADHB antibody images:
Western blot - HADHB antibody (ab88256)
All lanes : Anti-HADHB antibody (ab88256) at 1 µg/ml
Lane 1 : HADHB transfected 293T cell lysate
Lane 2 : Untransfected 293T cell lysate
Lysates/proteins at 25 µg per lane.
Secondary
Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution
Predicted band size : 51 kDa
Observed band size : 46 kDa (why is the actual band size different from the predicted?)
Western blot - HADHB antibody (ab88256)
Anti-HADHB antibody (ab88256) at 1 µg/ml + Human liver lysate at 25 µg
Secondary
Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution
Predicted band size : 51 kDa
Observed band size : 46 kDa (why is the actual band size different from the predicted?)
Western blot - HADHB antibody (ab88256)
Anti-HADHB antibody (ab88256) at 1 µg/ml + A431 cell lysate at 25 µg
Secondary
Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution
Predicted band size : 51 kDa
Observed band size : 48 kDa (why is the actual band size different from the predicted?)
References for Anti-HADHB antibody (ab88256)
ab88256 has not yet been referenced specifically in any publications.
Publishing research using ab88256? 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"