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HIBCH Polyclonal Antibody, HRP Conjugated

Applications

  • WB
  • ELISA
  • IHC-P
  • IHC-F

Predicted Reactivity

  • Human
Overview
Catalog # bs-15484R-HRP
Product Name HIBCH Polyclonal Antibody, HRP Conjugated
Applications
WB ELISA IHC-P IHC-F
Predicted Reactivity Human
Specifications
Conjugation HRP
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 (pH 7.4) 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
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500

Frequently asked questions

Which applications is HIBCH Polyclonal Antibody, HRP Conjugated (bs-15484R-HRP) validated for, and at what dilution?

bs-15484R-HRP is validated for WB (1:300-5000); ELISA (1:500-1000); IHC-P (1:200-400); IHC-F (1:100-500). These are recommended starting dilutions; optimal dilutions should be determined by the end user.

How should bs-15484R-HRP be stored?

Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

What immunogen was used to raise HIBCH Polyclonal Antibody, HRP Conjugated (bs-15484R-HRP)?

KLH conjugated synthetic peptide derived from human HIBCH