Anti-HADHA 抗体 (ab54477)
Key features and details
- Rabbit polyclonal to HADHA
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
製品の概要
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製品名
Anti-HADHA antibody
HADHA 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to HADHA -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Mouse, Rat, Human -
免疫原
Synthetic peptide surrounding amino acid 750 (Human)
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ポジティブ･コントロール
- Jurkat cell lysate 3T3 cell lysate Rat kidney lysate
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特記事項
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
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
バッファー
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 0.5% BSA, 30% Glycerol (glycerin, glycerine), PBS -
Concentration information loading... -
精製度
Protein A purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab54477の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| WB | (3) |
Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa).
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| 特記事項 |
|---|
|
WB
Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa). |
ターゲット情報
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機能
Bifunctional subunit. -
パスウェイ
Lipid metabolism; fatty acid beta-oxidation. -
関連疾患
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). -
配列類似性
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. -
細胞内局在
Mitochondrion. - Information by UniProt
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参照データベース
- 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 -
別名
- 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody
- 3 oxoacyl CoA thiolase antibody
- 78 kDa gastrin binding protein antibody
see all
画像
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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
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (44)
ab54477 は 44 報の論文で使用されています。
- 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