Rabbit Polyclonal HADHA antibody. Suitable for WB and reacts with Human, Mouse, Rat samples. Cited in 44 publications. Immunogen corresponding to Synthetic Peptide within Human HADHA.
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- Circulation research 128:1629-16412021NAD Repletion Reverses Heart Failure With Preserved Ejection Fraction.Applications:Unspecified applicationReactive species:Unspecified reactive speciesDan Tong,Gabriele G Schiattarella,Nan Jiang,Francisco Altamirano,Pamela A Szweda,Abdallah Elnwasany,Dong I Lee,Heesoo Yoo,David A Kass,Luke I Szweda,Sergio Lavandero,Eric Verdin,Thomas G Gillette,Joseph A HillPubMed 33882692
- Redox biology 41:1019232021Mitochondrial morphology, bioenergetics and proteomic responses in fatty acid oxidation disorders.Applications:Unspecified applicationReactive species:Unspecified reactive speciesSerena Raimo,Gabriella Zura-Miller,Hossein Fezelinia,Lynn A Spruce,Iordanis Zakopoulos,Al-Walid Mohsen,Jerry Vockley,Harry IschiropoulosPubMed 33725513
- Environmental toxicology 35:1033-10422020l-Carnitine protects against 1,4-benzoquinone-induced apoptosis and DNA damage by suppressing oxidative stress and promoting fatty acid oxidation in K562 cells.Applications:Unspecified applicationReactive species:Unspecified reactive speciesRongli Sun,Zhaodi Man,Jiahui Ji,Shuangbin Ji,Kai Xu,Yunqiu Pu,Linling Yu,Juan Zhang,Lihong Yin,Yuepu PuPubMed 32478940
- The EMBO journal 39:e1027312020CLUH granules coordinate translation of mitochondrial proteins with mTORC1 signaling and mitophagy.Applications:Unspecified applicationReactive species:Unspecified reactive speciesDavid Pla-Martín et. al.PubMed 32149416
- Journal of molecular and cellular cardiology 138:304-3172019Defining decreased protein succinylation of failing human cardiac myofibrils in ischemic cardiomyopathy.Applications:Unspecified applicationReactive species:Unspecified reactive speciesHadi R Ali,Cole R Michel,Ying H Lin,Timothy A McKinsey,Mark Y Jeong,Amrut V Ambardekar,Joseph C Cleveland,Richard Reisdorph,Nichole Reisdorph,Kathleen C Woulfe,Kristofer S FritzPubMed 31836543
- Nature communications 10:46712019TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes.Applications:Unspecified applicationReactive species:Unspecified reactive speciesJason W Miklas et. al.PubMed 31604922
- Scandinavian journal of medicine & science in sports 29:1881-18912019Effect of beta -adrenergic agonist and resistance training on maximal oxygen uptake and muscle oxidative enzymes in men.Applications:Unspecified applicationReactive species:Unspecified reactive speciesAnders Krogh Lemminger,Søren Jessen,Sajad Habib,Johan Onslev,Stella Feng Sheng Xu,Vibeke Backer,Jens Bangsbo,Morten HostrupPubMed 31442335
- American journal of physiology. Endocrinology and 317:E374-E3872019Linking bioenergetic function of mitochondria to tissue-specific molecular fingerprints.Applications:Unspecified applicationReactive species:Unspecified reactive speciesLisa Kappler et. al.PubMed 31211616
- Cell reports 26:3429-3443.e32019Multi-dimensional Transcriptional Remodeling by Physiological Insulin In Vivo.Applications:Unspecified applicationReactive species:Unspecified reactive speciesThiago M Batista,Ruben Garcia-Martin,Weikang Cai,Masahiro Konishi,Brian T O'Neill,Masaji Sakaguchi,Jong Hun Kim,Dae Young Jung,Jason K Kim,C Ronald KahnPubMed 30893613
- Physiological reports 7:e139762019Inclusion of sprints in moderate intensity continuous training leads to muscle oxidative adaptations in trained individuals.Applications:Unspecified applicationReactive species:Unspecified reactive speciesThomas P Gunnarsson,Nina Brandt,Matteo Fiorenza,Morten Hostrup,Henriette Pilegaard,Jens BangsboPubMed 30793541
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: PBS, 30% Glycerol (glycerin, glycerine), 0.5% BSA- Form
- Liquid
- Clonality
- Polyclonal
Immunogen
- Synthetic Peptide within Human HADHA. The exact immunogen used to generate this antibody is proprietary information. Database link P40939
Reactivity data
Select an application| WB | |
|---|---|
| Human | Tested |
| Mouse | Tested |
| Rat | Tested |
WB
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Human | Dilution info 0.5-4 µg/mL | Notes - |
Species Mouse | Dilution info 0.5-4 µg/mL | Notes - |
Species Rat | Dilution info 0.5-4 µg/mL | Notes - |
Associated Products
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Target data
Function
Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:1550553, PubMed:29915090, PubMed:30850536, PubMed:8135828). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:29915090, PubMed:30850536, PubMed:8135828). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787).
Alternative names
HADH, HADHA, 78 kDa gastrin-binding protein, Monolysocardiolipin acyltransferase, TP-alpha
Recommended products
Rabbit Polyclonal HADHA antibody. Suitable for WB and reacts with Human, Mouse, Rat samples. Cited in 44 publications. Immunogen corresponding to Synthetic Peptide within Human HADHA.
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: PBS, 30% Glycerol (glycerin, glycerine), 0.5% BSA- Form
- Liquid
- Clonality
- Polyclonal
- Immunogen
- Synthetic Peptide within Human HADHA. The exact immunogen used to generate this antibody is proprietary information. Database link P40939
- Purification technique
- Affinity purification Protein A
- Concentration
Storage
- Shipped at conditions
- Blue Ice
- Appropriate long-term storage conditions
- -20°C
- Aliquoting information
- Upon delivery aliquot
- Storage information
- Avoid freeze / thaw cycle
Notes
This product is manufactured by BioVision, an Abcam company and was previously called 3721 TFP1/HADHA Antibody.
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Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
The HADHA protein also called hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha is an essential component of the mitochondrial trifunctional protein complex. This protein has a molecular mass of about 79 kDa and is expressed mainly in tissues with high fatty acid oxidation rates like liver heart and muscle. HADHA plays a significant role in the beta-oxidation of long-chain fatty acids acting on hydroxyacyl-CoA substrates during this critical metabolic process.
- Biological function summary
HADHA is a part of the mitochondrial trifunctional protein complex which consists of four alpha and four beta subunits. It facilitates the hydration of enoyl-CoA to 3-hydroxyacyl-CoA and the subsequent dehydrogenation to 3-ketoacyl-CoA. This enzyme works closely with its partner the HADHB protein to carry out these reactions efficiently. These functions are important for energy production as they are steps in the breakdown of fatty acids necessary for ATP generation.
- Pathways
HADHA participates in the mitochondrial beta-oxidation pathway an essential pathway for energy production from fats. Alongside HADHB it catalyzes key reactions that allow the progressive shortening of fatty acid chains which further feeds into the citric acid cycle. This pathway links HADHA not only to HADHB but also to other enzymes involved in lipid metabolism and energy homeostasis including medium-chain specific acyl-CoA dehydrogenase (MCAD) reflecting its role in comprehensive metabolic networks.
- Associated diseases and disorders
Defects in HADHA are associated with mitochondrial trifunctional protein deficiency and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Both disorders disrupt normal fatty acid oxidation leading to a spectrum of symptoms including hypoketotic hypoglycemia and cardiomyopathy. These conditions highlight the relationship between HADHA and other proteins involved in fatty acid metabolism such as HADHB further highlighting their collective role in maintaining cellular energy balance.
Product promise
Tested
We have tested this species and application combination and it works. It is covered by our product promise.
Expected
We have not tested this specific species and application combination in-house, but expect it will work. It is covered by our product promise.
Predicted
This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
Not recommended
We do not recommend this combination. It is not covered by our product promise.
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2 product images
Western blot - Anti-HADHA antibody (ab54477)
HADHA Western blot staining using rabbit Anti-HADHA antibody
All lanes: Western blot - 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
SecondaryAll lanes: Anti-Rabbit IgG, HRP-Linked Antibody at 1/5000 dilution
Predicted band size: 83 kDa
Observed band size: 83 kDa
Image collected and cropped by CiteAb under a CC-BY license from the publication
Western blot - Anti-HADHA antibody (ab54477)
HADHA western blot using anti-HADHA antibody ab54477. Publication image and figure legend from Miklas, J. W., Clark, E., et al., 2019, Nat Commun, PubMed 31604922.
ab54477 was used in this publication in western blot. This may not be the same as the application(s) guaranteed by Abcam. For a full list of applications guaranteed by Abcam for ab54477 please see the product overview.
Generation of HADHA Mutant and Knockout stem cell derived cardiomyocytes. a Schematic of fatty acid beta-oxidation detailing the four enzymatic steps. b Schematic of HADHA KO DNA and protein sequence from WTC iPSC line showing a 22 bp deletion, which resulted in an early stop codon. c Schematic of HADHA Mut DNA and protein sequence from WTC iPSC line showing a 2 bp deletion and 9 bp insertion on the first allele and a 2 bp deletion on the second allele. RNA-Sequencing read counts show that the HADHA Mut expresses exons 4–20 resulting in a truncated protein. d Western analysis of HADHA expression and housekeeping protein β-Actin in WTC iPSCs. e Confocal microscopy of WT, HADHA Mut and HADHA KO hiPSC-CMs for the cardiac marker αActinin (green) and HADHA (red). f Seahorse analysis trace of fatty acid oxidation capacity of WT, HADHA Mut and HADHA KO hiPSC-CMs. n = 6–7 biological replicates. Source data are provided as a Source Data file
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