Anti-HADHA抗体(ab54477)
Key features and details
- Rabbit polyclonal to HADHA
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
概述
-
产品名称
Anti-HADHA抗体
参阅全部 HADHA 一抗 -
描述
兔多克隆抗体to HADHA -
宿主
Rabbit -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Mouse, Rat, Human -
免疫原
Synthetic peptide surrounding amino acid 750 (Human)
-
阳性对照
- Jurkat cell lysate 3T3 cell lysate Rat kidney lysate
-
常规说明
This product is manufactured by BioVision, an Abcam company and was previously called 3721 TFP1/HADHA Antibody.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
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: 0.01% Thimerosal (merthiolate)
Constituents: 0.5% BSA, 30% Glycerol (glycerin, glycerine), PBS -
Concentration information loading... -
纯度
Protein A purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
-
Compatible Secondaries
-
Isotype control
-
Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab54477于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。
| 应用 | Ab评论 | 说明 |
|---|---|---|
| WB | (3) |
Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa).
|
| 说明 |
|---|
|
WB
Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa). |
靶标
-
功能
Bifunctional subunit. -
通路
Lipid metabolism; fatty acid beta-oxidation. -
疾病相关
Defects in HADHA 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 enzyme activities of the TFP complex.
Defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016]. The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.
Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP) [MIM:609016]. AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome). -
序列相似性
In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.
In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family. -
细胞定位
Mitochondrion. - Information by UniProt
-
数据库链接
- Entrez Gene: 3030 Human
- Entrez Gene: 97212 Mouse
- Entrez Gene: 170670 Rat
- Omim: 600890 Human
- SwissProt: P40939 Human
- SwissProt: Q8BMS1 Mouse
- SwissProt: Q64428 Rat
- Unigene: 516032 Human
see all -
别名
- 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody
- 3 oxoacyl CoA thiolase antibody
- 78 kDa gastrin binding protein antibody
see all
图片
-
Western blot - Anti-HADHA antibody (ab54477)All lanes : Anti-HADHA antibody (ab54477) at 4 µg/ml
Lanes 1-2 : Jurkat cell lysate 30-50 ug/lane.
Lane 3 : 3T3 cell lysate 30-50 ug/lane.
Lane 4 : Rat kidney lysate 30-50 ug/lane.
Secondary
All lanes : Anti-Rabbit IgG, HRP-Linked Antibody at 1/5000 dilution
Predicted band size: 83 kDa
Observed band size: 83 kDa
实验方案
数据表及文件
-
SDS download
-
Datasheet download
文献 (44)
ab54477 被引用在 44 文献中.
- Raimo S et al. Mitochondrial morphology, bioenergetics and proteomic responses in fatty acid oxidation disorders. Redox Biol 41:101923 (2021). PubMed: 33725513
- Tong D et al. NAD+ Repletion Reverses Heart Failure With Preserved Ejection Fraction. Circ Res 128:1629-1641 (2021). PubMed: 33882692
- Pla-Martín D et al. CLUH granules coordinate translation of mitochondrial proteins with mTORC1 signaling and mitophagy. EMBO J 39:e102731 (2020). PubMed: 32149416
- Ali HR et al. Defining decreased protein succinylation of failing human cardiac myofibrils in ischemic cardiomyopathy. J Mol Cell Cardiol 138:304-317 (2020). PubMed: 31836543
- Sun R et al. l-Carnitine protects against 1,4-benzoquinone-induced apoptosis and DNA damage by suppressing oxidative stress and promoting fatty acid oxidation in K562 cells. Environ Toxicol 35:1033-1042 (2020). PubMed: 32478940