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HIBCH Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS627159

HIBCH is responsible for the specific hydrolysis of HIBYL-CoA, a valine catabolite, as well as the hydrolysis of beta-hydroxypropionyl-CoA, an intermediate in a minor pathway of propionate metabolism. More details

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Product Overview

Online Datasheet in PDF
Product Name
HIBCH, Polyclonal Antibody
Full Product Name
HIBCH (3-Hydroxyisobutyryl-coenzyme A Hydrolase, 3-Hydroxyisobutyryl-CoA Hydrolase Mitochondrial, HIB-CoA Hydrolase, HIBYLCOAH, HIBYL-CoA-H)
Product Gene Name
anti-HIBCH antibody
Product Synonym Names
Anti -HIBCH (3-Hydroxyisobutyryl-coenzyme A Hydrolase, 3-Hydroxyisobutyryl-CoA Hydrolase Mitochondrial, HIB-CoA Hydrolase, HIBYLCOAH, HIBYL-CoA-H)
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Form/Format
Supplied as a liquid in PBS, 0.09% sodium azide.
Purity/Purification
Purified
Purified by ammonium sulfate precipitation.
Specificity
Recognizes human HIBCH.
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Molecular Weight
43,482 Da
Chromosome Location
Chromosome: 2; NC_000002.11 (191069360..191184771, complement). Location: 2q32.2
OMIM
250620
3D Structure
ModBase 3D Structure for Q6NVY1
Immunogen
Synthetic peptide selected from the Center region of human HIBCH (KLH).

Product Categories/Family for anti-HIBCH antibody

Antibodies; Abs to Enzymes
Applications Tested/Suitable for anti-HIBCH antibody
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-HIBCH antibody
Suitable for use in ELISA, Western Blot, and Immunohistochemistry.
Dilution: ELISA: 1:1,000
Western Blot: 1:50-1:100

Western Blot (WB) of anti-HIBCH antibody

Western blot analysis of HIBCH Antibody (Center) in K562 cell line lysates (35ug/lane). HIBCH (arrow) was detected using the purified Pab.(2ug/ml)
Rabbit Anti-HIBCH Polyclonal Antibody – Cat# MBS627159

Immunohistochemistry (IHC) of anti-HIBCH antibody

Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with HIBCH antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.
Rabbit Anti-HIBCH Polyclonal Antibody, Immunohistochemistry (IHC) – Cat# MBS627159

Other Notes

Small volumes of anti-HIBCH antibody 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 HIBCH purchase

MBS627159 is an antibody targeting HIBCH. Also known as HIBCH (3-Hydroxyisobutyryl-coenzyme A Hydrolase, 3-Hydroxyisobutyryl-CoA Hydrolase Mitochondrial, HIB-CoA Hydrolase, HIBYLCOAH, HIBYL-CoA-H). Reactive in Human. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for HIBCH. It may not necessarily be applicable to this product.

NCBI GI #
52790425
NCBI GeneID
26275
UniProt Primary Accession #
Q6NVY1
UniProt Secondary Accession #
Q53GA8; Q53GF2; Q53RF7; Q53TC6; Q92931; Q9BS94; D3DPI4
UniProt Related Accession #
Q6NVY1
NCBI Official Full Name
HIBCH protein
NCBI Official Synonym Full Names
3-hydroxyisobutyryl-CoA hydrolase
NCBI Official Symbol
HIBCH
NCBI Official Synonym Symbols
HIBYLCOAH
UniProt Protein Name
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
UniProt Synonym Protein Names
3-hydroxyisobutyryl-coenzyme A hydrolase
Protein Family
3-hydroxyisobutyryl-CoA hydrolase
UniProt Gene Name
HIBCH
UniProt Entry Name
HIBCH_HUMAN
NCBI Protein Information
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIBYL-CoA-H; HIB-CoA hydrolase; OTTHUMP00000163512; OTTHUMP00000205554; 3-hydroxyisobutyryl-Coenzyme A hydrolase

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.

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

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
Show more

Research Articles on HIBCH

1. Observational study of gene-disease association. (HuGE Navigator)

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.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. 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, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Pathways associated with anti-HIBCH antibody

 Products by Pathway  Pathway Diagram
 Branched-chain Amino Acid Catabolism Pathway antibodies  Branched-chain Amino Acid Catabolism Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Propanoate Metabolism Pathway antibodies  Propanoate Metabolism Pathway Diagram
 Propanoate Metabolism Pathway antibodies  Propanoate Metabolism Pathway Diagram
 Valine, Leucine And Isoleucine Degradation Pathway antibodies  Valine, Leucine And Isoleucine Degradation Pathway Diagram
 Valine, Leucine And Isoleucine Degradation Pathway antibodies  Valine, Leucine And Isoleucine Degradation Pathway Diagram
 Beta-Alanine Metabolism Pathway antibodies  Beta-Alanine Metabolism Pathway Diagram
 Beta-Alanine Metabolism Pathway antibodies  Beta-Alanine Metabolism Pathway Diagram

Diseases associated with anti-HIBCH antibody

 Disease Name  Pubmed Publications
 Cardiovascular Diseases Antibodies  >1 publications with HIBCH and Cardiovascular Diseases
 BETA-HYDROXYISOBUTYRYL CoA DEACYLASE DEFICIENCY Antibodies  >1 publications with HIBCH and BETA-HYDROXYISOBUTYRYL CoA DEACYLASE DEFICIENCY

Organs/Tissues associated with anti-HIBCH antibody

 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >1 publications with HIBCH and Blood
 Ganglia Antibodies  >1 publications with HIBCH and Ganglia
 Nerve Antibodies  >1 publications with HIBCH and Nerve
 Muscle Antibodies  >1 publications with HIBCH and Muscle
 Skin Antibodies  >1 publications with HIBCH and Skin
 Brain Antibodies  >1 publications with HIBCH and Brain
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