Anti-HIBCH antibody (ab101672)
- 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
Recombinant fragment, corresponding to amino acids 98-358 of Human HIBCH (BC005190).
- Positive control
- HepG2 cell lysate, Human fetal brain, skeletal muscle, liver, kidney and heart tissue lysates. Human hepatocarcinoma tissue.
- 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
- Research Areas
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.
|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.|
- 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.
- 3 hydroxyisobutyryl Coenzyme A hydrolase antibody
- 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial antibody
- 3-hydroxyisobutyryl-CoA hydrolase antibody
- 3-hydroxyisobutyryl-coenzyme A hydrolase antibody
- BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibody
- HIB CoA hydrolase antibody
- HIB-CoA hydrolase antibody
- HIBCH antibody
- HIBCH_HUMAN antibody
- HIBCoA hydrolase antibody
- HIBYL CoA H antibody
- HIBYL CoAH antibody
- HIBYL-CoA-H antibody
- HIBYLCoA H antibody
- HIBYLCoAH antibody
- mitochondrial antibody
Anti-HIBCH antibody images
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.