JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB124585

Recombinant Human HIBCH protein

Be the first to review this product! Submit a review

|

(0 Publication)

Recombinant Human HIBCH protein is a Human Full Length protein, in the 33 to 386 aa range, expressed in Escherichia coli, with >90%, suitable for SDS-PAGE, Mass Spec.

View Alternative Names

3-hydroxyisobutyryl-coenzyme A hydrolase, HIB-CoA hydrolase, HIBYL-CoA-H, HIBCH

1 Images
SDS-PAGE - Recombinant Human HIBCH protein (AB124585)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human HIBCH protein (AB124585)

15% SDS-PAGE showing ab124585 at approximately 42.1kDa (3μg).

Key facts

Purity

>90% SDS-PAGE

Expression system

Escherichia coli

Tags

His tag N-Terminus

Applications

SDS-PAGE, Mass Spec

applications

Biologically active

No

Accession

Q6NVY1

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 10% Glycerol (glycerin, glycerine), 1.17% Sodium chloride, 0.32% Tris HCl, 0.02% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

storage-buffer

style
left-column

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "Mass Spec": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
style
left-column
style
left-column

Sequence info

[{"sequence":"MGSSHHHHHHSSGLVPRGSHMGSHMDAAEEVLLEKKGCTGVITLNRPKFLNALTLNMIRQIYPQLKKWEQDPETFLIIIKGAGGKAFCAGGDIRVISEAEKAKQKIAPVFFREEYMLNNAVGSCQKPYVALIHGITMGGGVGLSVHGQFRVATEKCLFAMPETAIGLFPDVGGGYFLPRLQGKLGYFLALTGFRLKGRDVYRAGIATHFVDSEKLAMLEEDLLALKSPSKENIASVLENYHTESKIDRDKSFILEEHMDKINSCFSANTVEEIIENLQQDGSSFALEQLKVINKMSPTSLKITLRQLMEGSSKTLQEVLTMEYRLSQACMRGHDFHEGVRAVLIDKDQSPKWKPADLKEVTEEDLNNHFKSLGSSDLKF","proteinLength":"Full Length","predictedMolecularWeight":"42.1 kDa","actualMolecularWeight":null,"aminoAcidEnd":386,"aminoAcidStart":33,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"Q6NVY1","tags":[{"tag":"His","terminus":"N-Terminus"}]}]
style
left-column

Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
style
left-column

Supplementary information

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

HIBCH or 3-hydroxyisobutyryl-CoA hydrolase is an enzyme involved in the catabolic pathway of valine metabolism. It exhibits a molecular mass of approximately 37 kDa. This enzyme processes 3-hydroxyisobutyryl-CoA into free 3-hydroxyisobutyrate and CoA. HIBCH is expressed in several tissues with high levels found in liver and kidney reflecting its active role in amino acid metabolism.
Biological function summary

The enzyme catalyzes a specific reaction in the branched-chain amino acid metabolic pathway which is an essential process for the degradation and utilization of valine. HIBCH functions independently not forming part of any larger complex. This enzyme is key for maintaining the balance of metabolites derived from valine and is involved in energy production from protein breakdown.

Pathways

HIBCH plays a significant role in the valine degradation pathway where it acts downstream of other enzymes like acyl-CoA dehydrogenase. During this pathway it collaborates with various enzymes to ensure efficient energy extraction from valine. This process links valine metabolism to the larger citric acid cycle where it coordinates with enzymes such as isovaleryl-CoA dehydrogenase.

Mutations or deficiencies in HIBCH correlate with 3-hydroxyisobutyryl-CoA hydrolase deficiency a metabolic disorder that can manifest as developmental delay neurological deficits or acute metabolic crises in severe cases. The enzyme has links to valine metabolism-related disorders where disturbances in its function can lead to elevated levels of neurotoxic intermediates. Its interaction with related enzymes such as branched-chain alpha-keto acid dehydrogenase further accentuates its importance in maintaining metabolic equilibrium.
style
left-column

Specifications

Form

Liquid

Additional notes

ab124585 is purified using conventional chromatography techniques.

style
left-column

General info

Function

Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.

Sequence similarities

Belongs to the enoyl-CoA hydratase/isomerase family.

Subcellular localisation

Mitochondrion

style
left-column

Product protocols

style
left-column

Target data

Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
See full target information HIBCH
style
left-column
style
left-column
style
right-column

Complementary Products

Primary Antibodies

AB153826

Anti-HIBCH antibody

Immunocytochemistry/ Immunofluorescence - Anti-HIBCH antibody (AB153826)

  • 0
  • 0
Primary Antibodies

AB32199

Anti-PTEN antibody [Y184]

20ul selling size|RabMAb|Recombinant|KO Validated|Trial Size

Western blot - Anti-PTEN antibody [Y184] (AB32199)

  • 5
  • 15
Primary Antibodies

AB207299

Anti-MMP7 antibody [EPR17888-71]

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMP7 antibody [EPR17888-71] (AB207299)

  • 0
  • 0
Primary Antibodies

AB109307

Anti-Axin 2 antibody [EPR2005(2)]

RabMAb|Recombinant|20ul selling size|Trial Size

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Axin 2 antibody [EPR2005(2)] (AB109307)

  • 4
  • 5

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