Anti-HADHA antibody (ab54477)
Key features and details
- Rabbit polyclonal to HADHA
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
Overview
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Product name
Anti-HADHA antibody
See all HADHA primary antibodies -
Description
Rabbit polyclonal to HADHA -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Mouse, Rat, Human -
Immunogen
Synthetic peptide surrounding amino acid 750 (Human)
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Positive control
- Jurkat cell lysate 3T3 cell lysate Rat kidney lysate
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General notes
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
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
Storage buffer
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 0.5% BSA, 30% Glycerol (glycerin, glycerine), PBS -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
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.
Application | Abreviews | Notes |
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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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Notes |
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WB
Use a concentration of 0.5 - 4 µg/ml. Detects a band of approximately 83 kDa (predicted molecular weight: 83 kDa). |
Target
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Function
Bifunctional subunit. -
Pathway
Lipid metabolism; fatty acid beta-oxidation. -
Involvement in disease
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). -
Sequence similarities
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. -
Cellular localization
Mitochondrion. - Information by UniProt
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Database links
- 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 -
Alternative names
- 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody
- 3 oxoacyl CoA thiolase antibody
- 78 kDa gastrin binding protein antibody
see all
Images
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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
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (44)
ab54477 has been referenced in 44 publications.
- 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