Mouse anti-Human/Mouse/Rat sapiens (Human) HIBCH Monoclonal Antibody
Mouse anti-Human/Mouse/Rat sapiens (Human) HIBCH Monoclonal Antibody - Image 2

Mouse anti-Human/Mouse/Rat sapiens (Human) HIBCH Monoclonal Antibody

Mouse anti-Human/Mouse/Rat sapiens (Human) HIBCH Monoclonal Antibody — Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite.

SKU: BCREC-000422MA Category:
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Product Specifications

Uniprot No.Q6NVY1
Target NamesHIBCH
Species ReactivityMouse
ImmunogenRecombinant Human HIBCH protein
Immunogen SpeciesHomo sapiens (Human)
ConjugateNon-conjugated
IsotypeMouse IgG2a
ClonalityMonoclonal
ApplicationsELISA, WB, FC
BufferPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
StorageUpon receipt, store at -20°C or -80°C. Avoid repeated freeze.

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.

Biological Context

Subcellular Location: Mitochondrion
Tissue Specificity: Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung
Disease Association: 3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD) : An autosomal recessive inborn error of valine metabolism. It causes severely delayed psychomotor development, neurodegeneration, increased lactic acid, and brain lesions in the basal ganglia. [The disease is caused by variants affecting the gene represented in this entry]
Pathway: Amino-acid degradation; L-valine degradation

Product Specifications

Mouse anti-Human/Mouse/Rat sapiens (Human) HIBCH Monoclonal Antibody is a recombinant protein. Suitable for ELISA and Western Blot applications.

SDS-PAGE: Single band at expected molecular weight confirming purity.

ELISA: Suitable as coating antigen or detection standard.

Western Blot: Compatible with standard Western Blot protocols.

Protein Interaction: Validated for SPR (Surface Plasmon Resonance) and BLI (Bio-Layer Interferometry) studies.

Shipping: Shipped at ambient temperature. Lyophilized protein is stable during transit.

Storage: Store lyophilized protein at -20°C to -80°C. Reconstituted protein should be aliquoted and stored at -80°C. Avoid repeated freeze-thaw cycles.

Shelf Life: 12 months from date of receipt when stored as recommended.

Shipping Time: Orders placed before 2 PM EST ship same day. International orders typically deliver within 5-10 business days.

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Protein Biology

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

Subcellular Location

Mitochondrion

Disease Association

3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD) : An autosomal recessive inborn error of valine metabolism. It causes severely delayed psychomotor development, neurodegeneration, increased lactic acid, and brain lesions in the basal ganglia. [The disease is caused by variants affecting the gene represented in this entry]

Tissue Specificity

Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung

Pathway

Amino-acid degradation; L-valine degradation

Gene: HIBCH  |  Organism: Homo sapiens  |  Synonyms: 3-hydroxyisobutyryl-coenzyme A hydrolase
Key Publications

Frequently Asked Questions

How do I order or inquire about this product?

Fill out the Online Inquiry form with your required quantity and specifications. You can also email sales@biocrestsci.com. Our team typically responds within 4 business hours with a quote and availability confirmation.

What is the shipping and delivery time?

Orders placed before 2 PM EST ship the same day. Domestic (US) delivery typically takes 2-3 business days. International orders deliver within 5-10 business days. All products are shipped at ambient temperature with appropriate packaging to ensure stability.

How should I store this recombinant protein?

Lyophilized proteins should be stored at -20°C to -80°C upon receipt. After reconstitution, aliquot and store at -80°C. Avoid repeated freeze-thaw cycles. Shelf life is 12 months from date of receipt when stored as recommended.

What quality controls are performed on your products?

Each product undergoes SDS-PAGE purity analysis (typically >85-95%), endotoxin testing, and bioactivity validation. Products are validated for ELISA, Western Blot, and SPR/BLI applications as specified on this product page. A Certificate of Analysis (CoA) is available upon request.

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