Overview
- Product nameAnti-HIBCH antibodySee all HIBCH primary antibodies ...
- DescriptionRabbit polyclonal to HIBCH
- Tested applicationsELISA, WB, IHC-P more details
- Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat - Immunogen
Recombinant fragment, corresponding to amino acids 98-358 of Human HIBCH (BC005190).
- Positive controlHepG2 cell lysate, Human fetal brain, skeletal muscle, liver, kidney and heart tissue lysates. Human hepatocarcinoma tissue.
Properties
- FormLiquid
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
- Storage bufferPreservative: 0.02% Sodium Azide
Constituents: PBS, pH 7.2 -
Concentration information loading... - PurityProtein A purified
- Clonality Polyclonal
- IsotypeIgG
- Research Areas
Applications
Our Abpromise guarantee covers the use of ab101672 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Notes |
|---|---|
| ELISA | ELISA: 1/20000 - 1/80000. |
| WB | WB: 1/1000 - 1/2000. Predicted molecular weight: 38 kDa. |
| IHC-P | IHC-P: 1/100 - 1/500. |
Target
- FunctionHydrolyzes 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.
- Tissue specificityHighly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
- PathwayAmino-acid degradation; L-valine degradation.
- Involvement in diseaseDefects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life.
- Sequence similaritiesBelongs to the enoyl-CoA hydratase/isomerase family.
- Cellular localizationMitochondrion.
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Database links
- Entrez Gene: 26275 Human
- Entrez Gene: 227095 Mouse
- Entrez Gene: 301384 Rat
- Omim: 610690 Human
- SwissProt: Q6NVY1 Human
- SwissProt: Q8QZS1 Mouse
- SwissProt: Q5XIE6 Rat
- Unigene: 656685 Human
- Unigene: 222063 Mouse
- Unigene: 8745 Rat
see all
Target information above from: UniProt accession
Q6NVY1
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010)
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Alternative names
- 3 hydroxyisobutyryl Coenzyme A hydrolase antibody3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial antibody3-hydroxyisobutyryl-CoA hydrolase antibody
- 3-hydroxyisobutyryl-coenzyme A hydrolase antibodyBETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibodyHIB CoA hydrolase antibodyHIB-CoA hydrolase antibodyHIBCH antibodyHIBCH_HUMAN antibodyHIBCoA hydrolase antibodyHIBYL CoA H antibodyHIBYL CoAH antibodyHIBYL-CoA-H antibodyHIBYLCoA H antibodyHIBYLCoAH antibodymitochondrial antibody
see all
Anti-HIBCH antibody images
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All lanes : Anti-HIBCH antibody (ab101672) at 1/1000 dilution
Lane 1 : HepG2 lysate
Lane 2 : Human fetal liver lysate
Lane 3 : Human fetal kidney lysate
Lane 4 : Human fetal heart lysate
Lane 5 : Human fetal brain lysate
Lane 6 : Human fetal skeletal muscle lysate
Predicted band size : 38 kDa -
ab101672 at 1/100 dilution, staining cytoplasmic HIBCH in formalin-fixed, paraffin-embedded Human hepatocarcinoma tissue, by Immunohistochemistry.
References for Anti-HIBCH antibody (ab101672)
ab101672 has not yet been referenced specifically in any publications.

