产品名称 Anti-HADHB抗体 参阅全部 HADHB 一抗
WB more details
Synthetic peptide corresponding to a region within C terminal amino acids 321-351 of Human HADHB, conjugated to KLH.
存放说明 Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C or -80°C. Avoid freeze / thaw cycle.
存储溶液 Preservative: 0.09% Sodium Azide
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纯度 Immunogen affinity purified
纯化说明 This antibody is purified through a protein A column, followed by peptide affinity purification.
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.
1/100 - 1/500. 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 (ab103174)参考文献
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"