製品の概要

  • 製品名Anti-HADHA antibody
    HADHA 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to HADHA
  • アプリケーション適用あり: IHC-P, ICC/IF, WBmore details
  • 種交差性
    交差種: Mouse, Rat, Human
  • 免疫原

    Synthetic peptide surrounding amino acid 750 (Human)

  • ポジティブ・コントロール
    • Jurkat cell lysate 3T3 cell lysate Rat kidney lysate

法規制情報

アプリケーション

Our Abpromise guarantee covers the use of ab54477 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
IHC-P Use a concentration of 1 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
ICC/IF Use a concentration of 1 µg/ml.
WB Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa).Can be blocked with HADHA peptide (ab155748).

ターゲット情報

  • 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
  • 参照データベース
  • 別名
    • 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody
    • 3 oxoacyl CoA thiolase antibody
    • 78 kDa gastrin binding protein antibody
    • 78 kDa gastrin-binding protein antibody
    • ECHA antibody
    • ECHA_HUMAN antibody
    • GBP antibody
    • HADH antibody
    • HADHA antibody
    • Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody
    • LCEH antibody
    • LCHAD antibody
    • Long chain 3-hydroxyacyl-CoA dehydrogenase antibody
    • Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit antibody
    • Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit antibody
    • Mitochondrial trifunctional enzyme alpha subunit antibody
    • Mitochondrial trifunctional protein alpha subunit antibody
    • MTPA antibody
    • Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody
    • TP ALPHA antibody
    • TP-alpha antibody
    • Trifunctional enzyme subunit alpha mitochondrial precursor antibody
    see all

Anti-HADHA antibody 画像

  • All lanes : Anti-HADHA antibody (ab54477) at 4 µg/ml

    Lane 1 : Jurkat cell lysate 30-50 ug/lane.
    Lane 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
    Anti-Rabbit IgG, HRP-Linked Antibody at 1/5000 dilution

    Predicted band size : 83 kDa
    Observed band size : 83 kDa
  • ICC/IF image of ab54477 stained HepG2 cells. The cells were 100% methanol fixed (5 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab54477, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

  • ab54477 (1µg/ml) staining HADHA in human ileum using an automated system (DAKO Autostainer Plus). Using this protocol there is strong mitochondrial staining.
    Sections were rehydrated and antigen retrieved with the Dako 3 in 1 AR buffer EDTA pH 9.0 in a DAKO PT Link. Slides were peroxidase blocked in 3% H2O2 in methanol for 10 mins. They were then blocked with Dako Protein block for 10 minutes (containing casein 0.25% in PBS) then incubated with primary antibody for 20 min and detected with Dako Envision Flex amplification kit for 30 minutes. Colorimetric detection was completed with Diaminobenzidine for 5 minutes. Slides were counterstained with Haematoxylin and coverslipped under DePeX. Please note that, for manual staining, optimization of primary antibody concentration and incubation time is recommended. Signal amplification may be required.

Anti-HADHA antibody (ab54477) 使用論文

This product has been referenced in:
  • Tripathi H  et al. Long-term preservation of donor corneas in glycerol for keratoplasty: exploring new protocols. Br J Ophthalmol 100:284-90 (2016). Read more (PubMed: 26508778) »
  • Wypijewski KJ  et al. Identification of caveolar resident proteins in ventricular myocytes using a quantitative proteomic approach: dynamic changes in caveolar composition following adrenoceptor activation. Mol Cell Proteomics 14:596-608 (2015). Read more (PubMed: 25561500) »

See all 11 Publications for this product

Product Wall

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (293T, HepG2, MEF)
Loading amount 30 µg
Specification 293T, HepG2, MEF
Gel Running Conditions Reduced Denaturing (8%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 7% · Temperature: 20°C
Username

Mr. Dongil Kim

Verified customer

投稿 Jan 14 2013

The reference number is ab155748.

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Mouse Tissue lysate - other (brain (mitochondria))
Loading amount 20 µg
Specification brain (mitochondria)
Gel Running Conditions Reduced Denaturing
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 20°C
Username

Abcam user community

Verified customer

投稿 Oct 09 2012

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Mouse Cell lysate - other (Mouse whole brain isolated Mitochondrial fraction)
Loading amount 15 µg
Specification Mouse whole brain isolated Mitochondrial fraction
Treatment Long Term Treatment A and B
Gel Running Conditions Reduced Denaturing (12% Criterion Gel from Bio-rad)
Blocking step Milk as blocking agent for 45 minute(s) · Concentration: 5% · Temperature: RT°C
Username

Dr. Jia Yao

Verified customer

投稿 Sep 10 2008

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"