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AB158632

Recombinant Human HADHB protein (GST tag N-Terminus)

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Recombinant Human HADHB protein (GST tag N-Terminus) is a Human Full Length protein, in the 1 to 474 aa range, expressed in Wheat germ, suitable for ELISA, WB.

View Alternative Names

MSTP029, HADHB, TP-beta

1 Images
SDS-PAGE - Recombinant Human HADHB protein (GST tag N-Terminus) (AB158632)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human HADHB protein (GST tag N-Terminus) (AB158632)

ab158632 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

P55084

Animal free

No

Carrier free

No

Species

Human

Storage buffer

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

storage-buffer

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Complementary Products

Primary Antibodies

AB230667

Anti-HADHB antibody

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-HADHB antibody (AB230667)

  • 0
  • 0
Primary Antibodies

AB233838

Anti-Citrate synthetase antibody [EPR8067] - BSA and Azide free

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Citrate synthetase antibody [EPR8067] - BSA and Azide free (AB233838)

  • 5
  • 4
Primary Antibodies

AB181114

Anti-CPT2 antibody [EPR13626] - C-terminal

RabMAb|Recombinant|KO Validated

Immunocytochemistry/ Immunofluorescence - Anti-CPT2 antibody [EPR13626] - C-terminal (AB181114)

  • 2
  • 1
Primary Antibodies

AB110296

Anti-ACADM/MCAD antibody [3B7BH7]

Immunocytochemistry/ Immunofluorescence - Anti-ACADM/MCAD antibody [3B7BH7] (AB110296)

  • 4
  • 1
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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>" } } }
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Sequence info

[{"sequence":"MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK","proteinLength":"Full Length","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":474,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"P55084","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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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
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Supplementary information

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

The protein HADHB also called the beta subunit of the Mitochondrial Trifunctional Protein is a part of the enzyme system that breaks down fatty acids. It has an approximate molecular mass of 51 kDa. This protein performs mechanical functions that include enoyl-CoA hydratase hydroxyacyl-CoA dehydrogenase and thiolase activities which facilitate the conversion of fatty acids into acetyl-CoA units. HADHB is expressed in tissues with high energy demands especially in the liver heart and skeletal muscle where it plays an essential role in energy production.
Biological function summary

The HADHB protein forms a complex with HADHA creating the Mitochondrial Trifunctional Protein. This complex is involved in the inner mitochondrial membrane's beta-oxidation of long-chain fatty acids. Long-chain fatty acids serve as a vital energy source and the HADHB-HADHA complex makes their metabolism more efficient. This process provides ATP essential for maintaining cellular energy homeostasis especially during fasting and intense exercise.

Pathways

HADHB participates in the fatty acid beta-oxidation pathway a critical component of lipid metabolism. Through this pathway HADHB interacts with proteins like ACADVL and ACADM which catalyze different stages of fatty acid breakdown. Beta-oxidation plays an important role in producing acetyl-CoA subsequently entering the citric acid cycle linking HADHB to energy metabolism. These interactions emphasize the importance of HADHB in maintaining metabolic balance within cells.

HADHB mutations can lead to mitochondrial trifunctional protein deficiency which results in metabolic disorders like fatty acid oxidation disorders and peripheral neuropathy. These conditions impair the body's ability to break down fatty acids affecting energy homeostasis. Disorders related to HADHB may also involve proteins such as CPT1A which transfers fatty acids into mitochondria highlighting the interconnectedness of these metabolic pathways. Understanding HADHB's role in these diseases enhances the potential for targeted therapeutic strategies.
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Specifications

Form

Liquid

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General info

Function

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (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 carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity (PubMed : 29915090, PubMed : 30850536, PubMed : 8135828).

Sequence similarities

Belongs to the thiolase-like superfamily. Thiolase family.

Subcellular localisation

Mitochondrion

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Product protocols

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Target data

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (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 carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity (PubMed : 29915090, PubMed : 30850536, PubMed : 8135828).
See full target information HADHB
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