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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: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol, 1% BSA, 1X PBS, pH 7.0
Concentration information loading...
Purity Immunogen affinity purified
Abpromise guarantee covers the use of
in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500 - 1/3000. Predicted molecular weight: 51 kDa.
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.
Information by UniProt
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
Anti-HADHB antibody images
References for Anti-HADHB antibody (ab96524)
has not yet been referenced specifically in any publications.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"