JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB54477

Anti-HADHA antibody

5

(3 Reviews)

|

(51 Publications)

Rabbit Polyclonal HADHA antibody. Suitable for WB and reacts with Human, Mouse, Rat samples. Cited in 51 publications. Immunogen corresponding to Synthetic Peptide within Human HADHA.

View Alternative Names

HADH, HADHA, 78 kDa gastrin-binding protein, Monolysocardiolipin acyltransferase, TP-alpha

2 Images
Western blot - Anti-HADHA antibody (AB54477)
  • WB

Supplier Data

Western blot - Anti-HADHA antibody (AB54477)

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

Secondary

All lanes:

Anti-Rabbit IgG, HRP-Linked Antibody at 1/5000 dilution

Predicted band size: 83 kDa

Observed band size: 83 kDa

false

Western blot - Anti-HADHA antibody (AB54477)
  • WB

CiteAb

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

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Mouse, Rat, Human

Applications

WB

applications

Immunogen

Synthetic Peptide within Human HADHA. The exact immunogen used to generate this antibody is proprietary information.

P40939

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "0.5-4 µg/mL", "WB-species-notes": "<p></p>" }, "Mouse": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "0.5-4 µg/mL", "WB-species-notes": "<p></p>" }, "Rat": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "0.5-4 µg/mL", "WB-species-notes": "<p></p>" } } }

Product details

This product is manufactured by BioVision, an Abcam company and was previously called 3721 TFP1/HADHA Antibody.

Properties and storage information

Form
Liquid
Purification technique
Affinity purification Protein A
Storage buffer
Preservative: 0.01% Thimerosal (merthiolate) Constituents: PBS, 30% Glycerol (glycerin, glycerine), 0.5% BSA
Shipped at conditions
Blue Ice
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

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.

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 protocols

For this product, it's our understanding that no specific protocols are required. You can visit:

Target data

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).
See full target information HADHA

Publications (51)

Recent publications for all applications. Explore the full list and refine your search

Scandinavian journal of medicine & science in sports 34:e14768 PubMed39604207

2024

Exercise Training Counteracts Compromised Mitochondrial Capacity Induced by Energy Restriction in Prediabetics in a Sex-Dependent Manner.

Applications

Unspecified application

Species

Unspecified reactive species

Magni Mohr,Jerónimo Aragón Vela,May-Britt Skoradal,Martin Thomassen,Søren Andersen Skriver,Mette Hansen,Ioannis G Fatouros,Peter Krustrup,Nikolai B Nordsborg

Scientific reports 14:12521 PubMed38822085

2024

Regulation of TSC2 lysosome translocation and mitochondrial turnover by TSC2 acetylation status.

Applications

Unspecified application

Species

Unspecified reactive species

Patricia Marqués,Jesús Burillo,Carlos González-Blanco,Beatriz Jiménez,Gema García,Ana García-Aguilar,Sarai Iglesias-Fortes,Ángela Lockwood,Carlos Guillén

Alzheimer's & dementia : the journal of the Alzheimer's Association 20:1637-1655 PubMed38055782

2023

Early-life stress and amyloidosis in mice share pathogenic pathways involving synaptic mitochondria and lipid metabolism.

Applications

Unspecified application

Species

Unspecified reactive species

Janssen M Kotah,Mandy S J Kater,Niek Brosens,Sylvie L Lesuis,Roberta Tandari,Thomas M Blok,Luca Marchetto,Ella Yusaf,Frank T W Koopmans,August B Smit,Paul J Lucassen,Harm J Krugers,Mark H G Verheijen,Aniko Korosi

Scandinavian journal of medicine & science in sports 32 Suppl 1:39-53 PubMed34427373

2021

Skeletal muscle phenotype and game performance in elite women football players.

Applications

Unspecified application

Species

Unspecified reactive species

Magni Mohr,Ioannis G Fatouros,Athanasios Z Jamurtas,Dimitrios Draganidis,Martin Thomassen,Christina Ørntoft,Georgios Ermidis,Georgios Loules,Dimitrios Batsilas,Athanasios Poulios,Konstantinos Papanikolaou,Morten B Randers,Peter Krustrup,Lars Nybo

Circulation research 128:1629-1641 PubMed33882692

2021

NAD Repletion Reverses Heart Failure With Preserved Ejection Fraction.

Applications

Unspecified application

Species

Unspecified reactive species

Dan 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 Hill

Redox biology 41:101923 PubMed33725513

2021

Mitochondrial morphology, bioenergetics and proteomic responses in fatty acid oxidation disorders.

Applications

Unspecified application

Species

Unspecified reactive species

Serena Raimo,Gabriella Zura-Miller,Hossein Fezelinia,Lynn A Spruce,Iordanis Zakopoulos,Al-Walid Mohsen,Jerry Vockley,Harry Ischiropoulos

Environmental toxicology 35:1033-1042 PubMed32478940

2020

l-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 application

Species

Unspecified reactive species

Rongli Sun,Zhaodi Man,Jiahui Ji,Shuangbin Ji,Kai Xu,Yunqiu Pu,Linling Yu,Juan Zhang,Lihong Yin,Yuepu Pu

The EMBO journal 39:e102731 PubMed32149416

2020

CLUH granules coordinate translation of mitochondrial proteins with mTORC1 signaling and mitophagy.

Applications

Unspecified application

Species

Unspecified reactive species

David Pla-Martín,Désirée Schatton,Janica L Wiederstein,Marie-Charlotte Marx,Salim Khiati,Marcus Krüger,Elena I Rugarli

Journal of molecular and cellular cardiology 138:304-317 PubMed31836543

2019

Defining decreased protein succinylation of failing human cardiac myofibrils in ischemic cardiomyopathy.

Applications

Unspecified application

Species

Unspecified reactive species

Hadi 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 Fritz

Nature communications 10:4671 PubMed31604922

2019

TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes.

Applications

Unspecified application

Species

Unspecified reactive species

Jason W Miklas,Elisa Clark,Shiri Levy,Damien Detraux,Andrea Leonard,Kevin Beussman,Megan R Showalter,Alec T Smith,Peter Hofsteen,Xiulan Yang,Jesse Macadangdang,Tuula Manninen,Daniel Raftery,Anup Madan,Anu Suomalainen,Deok-Ho Kim,Charles E Murry,Oliver Fiehn,Nathan J Sniadecki,Yuliang Wang,Hannele Ruohola-Baker
View all publications

Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.
For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com