HIBCH Polyclonal Antibody, AbBy Fluor-350 Conjugated
Applications
Predicted Reactivity
| Overview | |
| Catalog # | bs-15484R-BF350-100uL |
| Product Name | HIBCH Polyclonal Antibody, AbBy Fluor-350 Conjugated |
| Applications |
WB
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Predicted Reactivity | Human |
| Specifications | |
| Conjugation | AbBy Fluor-350 |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human HIBCH |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Gene ID | 26275 |
| Subcellular location | Cytoplasm |
| Synonyms | 3 hydroxyisobutyryl Coenzyme A hydrolase; 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial; HIBCH_HUMAN. |
| Background | HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and _-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of _-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia. |
| Application Dilution | |
| WB | 1:300-5000 |
| IF(IHC-P) | 1:50-200 |
| IF(IHC-F) | 1:50-200 |
| IF(ICC) | 1:50-200 |
Frequently asked questions
Which applications is HIBCH Polyclonal Antibody, AbBy Fluor-350 Conjugated (bs-15484R-BF350) validated for, and at what dilution?
bs-15484R-BF350 is validated for WB (1:300-5000); IF(IHC-P) (1:50-200); IF(IHC-F) (1:50-200); IF(ICC) (1:50-200). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
How should bs-15484R-BF350 be stored?
Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise HIBCH Polyclonal Antibody, AbBy Fluor-350 Conjugated (bs-15484R-BF350)?
KLH conjugated synthetic peptide derived from human HIBCH
What are the excitation and emission wavelengths of the AbBy Fluor-350 conjugate (bs-15484R-BF350)?
AbBy Fluor-350 on bs-15484R-BF350: excitation / emission 346nm/442nm.