概述
-
产品名称Anti-HADHB抗体
参阅全部 HADHB 一抗 -
描述山羊多克隆抗体to HADHB
-
宿主Goat
-
经测试应用适用于: WBmore details
-
种属反应性与反应: Human
-
免疫原
Synthetic peptide: TILTYPFKNLPT, corresponding to N terminal amino acids 2-13 of Human HADHB.
-
阳性对照
- Human brain and Jurkat extracts.
-
常规说明
The HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy. Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.
性能
-
形式Liquid
-
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: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris-saline. pH 7.3 -
Concentration information loading... -
纯度Immunogen affinity purified
-
纯化说明Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
-
Primary antibody说明The HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy. Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.
-
克隆多克隆
-
同种型IgG
-
研究领域
相关产品
-
Compatible Secondaries
-
Immunizing Peptide (Blocking)
应用
Our Abpromise guarantee covers the use of ab10093 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| 应用 | Ab评论 | 说明 |
|---|---|---|
| WB | Use at an assay dependent concentration. Can be blocked with Human HADHB peptide (ab23180). |
靶标
-
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.
-
细胞定位Mitochondrion.
- Information by UniProt
-
数据库链接
- Entrez Gene: 3032 Human
- Omim: 143450 Human
- SwissProt: P55084 Human
- Unigene: 515848 Human
-
别名
- 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
文献
ab10093 has not yet been referenced specifically in any publications.
客户评价及客户问答
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"