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HIBCH elisa kit :: Porcine 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH) ELISA Kit

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Catalog # MBS7223709
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HIBCH elisa kit
Product Name

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH), ELISA Kit

Full Product Name

Porcine 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH) ELISA Kit

Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Request for Current Manual Insert
OMIM
610690
Species Reactivity
Samples
Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
Preparation and Storage
Store all reagents at 2-8 degree C.
Sample Preparation
We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team
Product Note
Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.
Other Notes
Small volumes of HIBCH elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Searchable Terms for HIBCHpurchase
MBS7223709 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing HIBCH. The ELISA analytical biochemical technique of the MBS7223709 kit is based on HIBCH antibody-HIBCH antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HIBCH antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HIBCH. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
Product Categories/Family for HIBCH elisa kit
NCBI/Uniprot data below describe general gene information for HIBCH. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
38,012 Da
NCBI Official Full Name
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial isoform 1
NCBI Official Synonym Full Names
3-hydroxyisobutyryl-CoA hydrolase
NCBI Official Symbol
HIBCH  [Similar Products]
NCBI Official Synonym Symbols
HIBYLCOAH
  [Similar Products]
NCBI Protein Information
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIBYL-CoA-H; HIB-CoA hydrolase; 3-hydroxyisobutyryl-Coenzyme A hydrolase
UniProt Protein Name
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
UniProt Synonym Protein Names
3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H
UniProt Gene Name
HIBCH  [Similar Products]
UniProt Synonym Gene Names
HIB-CoA hydrolase; HIBYL-CoA-H  [Similar Products]
UniProt Entry Name
HIBCH_HUMAN
NCBI Summary for HIBCH
This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]
UniProt Comments for HIBCH
HIBCH: Hydrolyzes 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. Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD); 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. Belongs to the enoyl-CoA hydratase/isomerase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Hydrolase; Carbohydrate Metabolism - propanoate; Other Amino Acids Metabolism - beta-alanine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 3.1.2.4

Chromosomal Location of Human Ortholog: 2q32.2

Cellular Component: mitochondrial matrix

Molecular Function: 3-hydroxyisobutyryl-CoA hydrolase activity

Biological Process: valine catabolic process; branched chain family amino acid catabolic process

Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Products associated with HIBCH elisa kit
 Reference Product  PubMed Publications
 ECHS1 elisa kit  >1 publications with HIBCH and ECHS1
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