JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB158631

Recombinant Human HADHA protein

Be the first to review this product! Submit a review

|

(0 Publication)

Recombinant Human HADHA protein is a Human Full Length protein, in the 1 to 763 aa range, expressed in Wheat germ, suitable for ELISA, WB.

View Alternative Names

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

1 Images
SDS-PAGE - Recombinant Human HADHA protein (AB158631)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human HADHA protein (AB158631)

ab158631 on a 12.5% SDS-PAGE stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

ELISA, WB

applications

Biologically active

No

Accession

P40939

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }

Sequence info

[{"sequence":"MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ","proteinLength":"Full Length","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":763,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"P40939","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]

Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False

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.

Specifications

Form

Liquid

General info

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).

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.

Subcellular localisation

Mitochondrion

Product protocols

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

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