HIBCH antibody (AA 251-350) (AbBy Fluor® 488)

Catalog No. ABIN1694581

Product Details anti-HIBCH Antibody (AbBy Fluor® 488)

Target: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Binding Specificity: AA 251-350

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HIBCH antibody is conjugated to AbBy Fluor® 488

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HIBCH

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for HIBCH

Target: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Alternative Name: Hibch (HIBCH Products)

Background:

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.

Gene ID: 26275

Pathways: Monocarboxylic Acid Catabolic Process