存放说明 Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
存储溶液 Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol, 1% BSA, 1X PBS, pH 7.0
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纯度 Immunogen affinity purified
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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.
1/500 - 1/3000. Predicted molecular weight: 51 kDa.
通路 Lipid metabolism; fatty acid beta-oxidation.
疾病相关 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.
序列相似性 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 (ab96524)参考文献
has not yet been referenced specifically in any publications.
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