Synthetic peptide corresponding to a region within C terminal amino acids 321-351 of Human HADHB, conjugated to KLH.
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Preservative: 0.09% Sodium Azide
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Immunogen affinity purified
This antibody is purified through a protein A column, followed by peptide affinity purification.
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in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/100 - 1/500. Predicted molecular weight: 51 kDa.
Lipid metabolism; fatty acid beta-oxidation.
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.
Belongs to the thiolase family.
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 (ab103174)参考文献
has not yet been referenced specifically in any publications.
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