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.

Applications: ELISA, WB, FC

Biological Activity: Not implied unless activity data are explicitly listed on this product page, the datasheet, or the CoA.

Storage: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Shipping & Lead Time: Availability, shipping conditions, and estimated lead time are confirmed in the formal quotation or order confirmation based on the product format, destination, and order requirements.

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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
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Frequently Asked Questions

How do I order or inquire about this product?

Submit the Online Inquiry form with your required quantity and specifications, or email sales@biocrestsci.com. Available sizes, pricing, stock status, documentation, and estimated lead time are confirmed in the formal quotation.

What is the shipping and delivery time?

Availability, dispatch timing, shipping conditions, and delivery estimates depend on the product format, destination, and order requirements. These details are confirmed in the formal quotation or order confirmation.

How should this product be stored?

Follow the storage and handling instructions in the product-specific datasheet and Certificate of Analysis (CoA). Requirements may vary by product form and formulation; contact BioCrest Sci if product-specific guidance is needed.

What quality controls are performed on your products?

Quality-control data such as identity, purity, endotoxin level, or biological activity are provided only when specified on this product page, the datasheet, or Certificate of Analysis (CoA). Biological activity is not implied unless explicitly stated.

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