HIBCH Rabbit Monoclonal Antibody for WB, IHC-P, ELISA - Q6NVY1

Core Product Specifications and Parameters

Biological Background: HIBCH Function and Localization

• HIBCH, also known as 3-hydroxyisobutyryl-CoA hydrolase (HIBYL-CoA-H), is a mitochondrial enzyme that hydrolyzes 3-hydroxyisobutyryl-CoA, a key step in valine catabolism.
• It also shows high activity toward isobutyryl-CoA and can hydrolyze 3-hydroxypropanoyl-CoA.
• HIBCH is localized in the mitochondrion, where it functions in branched-chain amino acid degradation.
• The protein has a molecular weight of approximately 43 kDa and undergoes post-translational modifications including acetylation and phosphorylation.
• Expressed at high levels in liver and kidney, with lower expression in heart, muscle, and brain; not detected in lung.
• Mutations in HIBCH are associated with metabolic disease variants, highlighting its clinical relevance in inborn errors of metabolism.

Experimental Guidance and Technical Tips

• The immunogen covers amino acids 33-330 of the mature mitochondrial protein; consider validating antibody recognition of endogenous HIBCH in relevant sample systems (e.g., liver or kidney lysates).
• For Western blotting, a band at approximately 43 kDa is expected; mitochondrial enrichment may improve detection sensitivity.
• In IHC-P, include liver or kidney tissue sections as positive controls, given high HIBCH expression at the protein level.
• This antibody can be employed in ELISA formats; confirm linearity and specificity using recombinant protein or characterized biological samples.

CamelBio: Your One-Stop Sourcing Bridge

As your one-stop IVD raw-material partner, CamelBio streamlines access to high-quality antibodies, antigens, and ancillary reagents for metabolic and mitochondrial research. For HIBCH and other valine catabolism targets, we offer validated monoclonal/polyclonal antibodies, matched antibody pairs, and custom bulk sourcing to support your diagnostic development from concept to clinic.