Human HIBCH Recombinant Protein – Cat# MBS144549

3-Hydroxyisobutyryl-Coa Hydrolase (HIBCH) Human Recombinant Protein

Name: HIBCH Recombinant Protein; Human; MyBioSource
Brand: MyBioSource
SKU: MBS144549
Price: 250 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS144549

HIBCH Recombinant produced in E. coli is a single polypeptide chain containing 379 amino acids (33-386) and having a molecular mass of 42.1kDa.HIBCH is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. More details

$250 / 0.002 mg +1 FREE 8GB USB

Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

3-Hydroxyisobutyryl-CoA Hydrolase (HIBCH), Recombinant Protein

Full Product Name

Recombinant Human 3-Hydroxyisobutyryl-CoA Hydrolase

Product Gene Name

HIBCH recombinant protein

Product Synonym Names

HIBCH Human; 3-Hydroxyisobutyryl-CoA Hydrolase Human Recombinant; 3-hydroxyisobutyryl-coenzyme A hydrolase mitochondrial; HIBYL-CoA-H; HIB-CoA hydrolase; EC 3.1.2.4

Sequence

MGSSHHH HHH SSGLVPRGSH MGSHMD AAEE VLLEKKGCTG VITLNRPKFL NALTLNMIRQ IYPQLKKWEQ DPETFLIIIK GAGGKAFCAG GDIRVISEAE KAKQKIAPVF FREEYMLNNA VGSCQKPYVA LIHGITMGGG VGLSVHGQFR VATEKCLFAM PETAIGLFPD VGGGYFLPRL QGKLGYFLAL TGFRLKGRDV YRAGIATHFV DSEKLAMLEE DLLALKSPSK ENIASVLENY HTESKIDRDK SFILEEHMDK INSCFSANTV EEIIENLQQD GSSFALEQLK VINKMSPTSL KITLRQLMEG SSKTLQEVLT MEYRLSQACM RGHDFHEGVR AVLIDKDQSP KWKPADLKEV TEEDLNNHFK SLGSSDLKF.

Form/Format

The HIBCH solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 200mM Nacl, 1mM DTT and 10% glycerol.

Purity/Purification

Greater than 90% as determined by SDS-PAGE.

Preparation and Storage

Store at 4°C if entire vial will be used within 2-4 weeks.
Store, frozen at -20°C for longer periods of time.
For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).

Avoid multiple freeze-thaw cycles.

OMIM

250620

3D Structure

ModBase 3D Structure for Q6NVY1

Source

E.coli

Physical Appearance

Sterile filtered colorless solution

Product Categories/Family for HIBCH recombinant protein

Enzymes; Hydrolase

Other Notes

Small volumes of HIBCH recombinant protein 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

MBS144549 is a recombinant protein targeting HIBCH. Also known as Recombinant Human 3-Hydroxyisobutyryl-CoA Hydrolase.

Additional Details

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

NCBI GI #

37594471

NCBI GeneID

26275

NCBI Accession #

NP_055177.2

UniProt Primary Accession #

Q6NVY1

UniProt Secondary Accession #

Q53GA8; Q53GF2; Q53RF7; Q53TC6; Q92931; Q9BS94; D3DPI4

UniProt Related Accession #

Q6NVY1

GenBank Nucleotide #

NM_014362.3

NCBI Official Full Name

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial isoform 1

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; HIB-CoA hydrolase; HIBYL-CoA-H

Protein Family

3-hydroxyisobutyryl-CoA hydrolase

UniProt Gene Name

HIBCH

UniProt Synonym Gene Names

HIB-CoA hydrolase; HIBYL-CoA-H

UniProt Entry Name

HIBCH_HUMAN

NCBI Protein Information

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-Coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H

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

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

Research Articles on HIBCH

1. findings demonstrated a novel homozygous pathogenic missense mutation c.950G <A; p.Gly317Glu in the HIBCH gene, which segregated with infantile-onset neurodegeneration within a Pakistani family; HIBCH deficiency, a disorder of valine catabolism, is a novel cause of the multiple mitochondrial dysfunctions syndrome

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

Products associated with HIBCH recombinant protein

Reference Product	PubMed Publications
ECHS1 recombinant protein	>1 publications with HIBCH and ECHS1

Pathways associated with HIBCH recombinant protein

Products by Pathway	Pathway Diagram
Branched-chain Amino Acid Catabolism Pathway antibodies	Branched-chain Amino Acid Catabolism Pathway Diagram
Carbon Metabolism Pathway antibodies	Carbon Metabolism Pathway Diagram
Carbon Metabolism Pathway antibodies	Carbon Metabolism Pathway Diagram
Malonate Semialdehyde Pathway, Propanoyl-CoA => Acetyl-CoA antibodies	Malonate Semialdehyde Pathway, Propanoyl-CoA => Acetyl-CoA Diagram
Malonate Semialdehyde Pathway, Propanoyl-CoA => Acetyl-CoA antibodies	Malonate Semialdehyde Pathway, Propanoyl-CoA => Acetyl-CoA Diagram
Metabolic Pathways antibodies	Metabolic Pathways Diagram
Metabolism Pathway antibodies	Metabolism Pathway 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

Diseases associated with HIBCH recombinant protein

Disease Name	Pubmed Publications
Beta-Hydroxyisobutyryl CoA Deacylase Deficiency Antibodies	>4 publications with HIBCH and Beta-Hydroxyisobutyryl CoA Deacylase Deficiency
Nervous System Diseases Antibodies	>3 publications with HIBCH and Nervous System Diseases
Mitochondrial Diseases Antibodies	>2 publications with HIBCH and Mitochondrial Diseases
Nerve Degeneration Antibodies	>2 publications with HIBCH and Nerve Degeneration
Prostatic Neoplasms Antibodies	>1 publications with HIBCH and Prostatic Neoplasms
Cognition Disorders Antibodies	>1 publications with HIBCH and Cognition Disorders
Atrophy Antibodies	>1 publications with HIBCH and Atrophy
Hypertrophy Antibodies	>1 publications with HIBCH and Hypertrophy
Cardiovascular Diseases Antibodies	>1 publications with HIBCH and Cardiovascular Diseases
Liver Diseases Antibodies	>1 publications with HIBCH and Liver Diseases

Organs/Tissues associated with HIBCH recombinant protein

Organ/Tissue Name	Pubmed Publications
Brain Antibodies	>5 publications with HIBCH and Brain
Nerve Antibodies	>3 publications with HIBCH and Nerve
Ganglia Antibodies	>3 publications with HIBCH and Ganglia
Muscle Antibodies	>3 publications with HIBCH and Muscle
Blood Antibodies	>3 publications with HIBCH and Blood
Skin Antibodies	>2 publications with HIBCH and Skin
Prostate Antibodies	>1 publications with HIBCH and Prostate