Human IVD Recombinant Protein – Cat# MBS143650

Isovaleryl Coenzyme A Dehydrogenase (IVD) Human Recombinant Protein

Name: IVD Recombinant Protein; Human; MyBioSource
Brand: MyBioSource
SKU: MBS143650
Price: 250 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS143650

Description: IVD Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 415 amino acids (33-426 a.a.) and having a molecular mass of 45.3 kDa. The IVD is fused to a 20 amino acid his tag at N-terminus and purified by… More details

$250 / 0.005 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

Isovaleryl Coenzyme A Dehydrogenase (IVD), Recombinant Protein

Full Product Name

Recombinant Human Isovaleryl Coenzyme A Dehydrogenase

Product Gene Name

IVD recombinant protein

Product Synonym Names

IVD Human; Isovaleryl Coenzyme A Dehydrogenase Human Recombinant; FLJ12715; isovaleryl-CoA dehydrogenase mitochondrial; FLJ34849; EC 1.3.99.10; IVD; ACAD2

Sequence

MGSSHHH HHH SSGLVPRGSH MHSLLPVDDA INGLSEEQRQ LRQTMAKFLQ EHLAPKAQEI DRSNEFKNLR EFWKQLGNLG VLGITAPVQY GGSGLGYLEH VLVMEEISRA SGAVGLSYGA HSNLCINQLV RNGNEAQKEK YLPKLISGEY IGALAMSEPN AGSDVVSMKL KAEKKGNHYI LNGNKFWITN GPDADVLIVY AKTDLAAVPA SRGITAFIVE KGMPGFSTSK KLDKLGMRGS NTCELIFEDC KIPAANILGH ENKGVYVLMS GLDLERLVLA GGPLGLMQAV LDHTIPYLHV REAFGQKIGH FQLMQGKMAD MYTRLMACRQ YVYNVAKACD EGHCTAKDCA GVILYSAECA TQVALDGIQC FGGNGYINDF PMGRFLRDAK LYEIGAGTSE VRRLVIGRAF NADFH

Host

E Coli

Form/Format

The IVD protein solution contains 20mM Tris-HCl pH-8, 1mM DTT and 10% glycerol.
Sterile Filtered colorless solution.

Purity/Purification

Greater than 90.0% as determined by SDS-PAGE.

Preparation and Storage

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree 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.

Molecular Weight

42,724 Da

OMIM

243500

3D Structure

ModBase 3D Structure for P26440

Product Categories/Family for IVD recombinant protein

Enzymes; Dehydrogenase

Other Notes

Small volumes of IVD 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 IVD purchase

MBS143650 is a recombinant protein targeting IVD. Also known as Recombinant Human Isovaleryl Coenzyme A Dehydrogenase. Host: E Coli.

Additional Details

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

NCBI GI #

226958414

NCBI GeneID

3712

NCBI Accession #

NP_001152980.1

UniProt Primary Accession #

P26440

UniProt Secondary Accession #

Q53XZ9; Q96AF6; B2RCV5; B3KVI7; J3KR54

UniProt Related Accession #

P26440

GenBank Nucleotide #

NM_001159508.1

NCBI Official Full Name

isovaleryl-CoA dehydrogenase, mitochondrial isoform 2

NCBI Official Synonym Full Names

isovaleryl-CoA dehydrogenase

NCBI Official Symbol

IVD

NCBI Official Synonym Symbols

ACAD2

UniProt Protein Name

Isovaleryl-CoA dehydrogenase, mitochondrial

Protein Family

Isovaleryl-CoA dehydrogenase

UniProt Gene Name

IVD

UniProt Synonym Gene Names

IVD

UniProt Entry Name

IVD_HUMAN

NCBI Protein Information

isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase

NCBI Summary for IVD

Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]

UniProt Comments for IVD

IVD: Defects in IVD are the cause of isovaleric acidemia (IVA). IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.4; Mitochondrial

Chromosomal Location of Human Ortholog: 15q14-q15

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix

Molecular Function: isovaleryl-CoA dehydrogenase activity; FAD binding

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

Disease: Isovaleric Acidemia

Research Articles on IVD

1. study reports the first Saudi isovaleric acidemia patients from a consanguineous family with a novel transversion (p.G362V) and briefly discuss likely phenotype-genotype correlation of the disease in the Saudi population

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 IVD recombinant protein

Reference Product	PubMed Publications
BCKDHA recombinant protein	>1 publications with IVD and BCKDHA
DBT recombinant protein	>1 publications with IVD and DBT

Pathways associated with IVD recombinant protein

Products by Pathway	Pathway Diagram
Branched-chain Amino Acid Catabolism Pathway antibodies	Branched-chain Amino Acid Catabolism Pathway Diagram
Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies	Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies	Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway 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
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
Leucine Degradation Pathway antibodies	Leucine Degradation Pathway Diagram

Diseases associated with IVD recombinant protein

Disease Name	Pubmed Publications
Disease Models, Animal Antibodies	>85 publications with IVD and Disease Models, Animal
Death Antibodies	>77 publications with IVD and Death
Inflammation Antibodies	>76 publications with IVD and Inflammation
Cardiovascular Diseases Antibodies	>74 publications with IVD and Cardiovascular Diseases
Necrosis Antibodies	>68 publications with IVD and Necrosis
Mental Disorders Antibodies	>54 publications with IVD and Mental Disorders
Brain Diseases Antibodies	>46 publications with IVD and Brain Diseases
Heart Diseases Antibodies	>29 publications with IVD and Heart Diseases
Nerve Degeneration Antibodies	>27 publications with IVD and Nerve Degeneration
Fibrosis Antibodies	>26 publications with IVD and Fibrosis

Organs/Tissues associated with IVD recombinant protein

Organ/Tissue Name	Pubmed Publications
Bone Antibodies	>647 publications with IVD and Bone
Blood Antibodies	>183 publications with IVD and Blood
Connective Tissue Antibodies	>64 publications with IVD and Connective Tissue
Brain Antibodies	>55 publications with IVD and Brain
Nerve Antibodies	>51 publications with IVD and Nerve
Bone Marrow Antibodies	>46 publications with IVD and Bone Marrow
Heart Antibodies	>38 publications with IVD and Heart
Muscle Antibodies	>33 publications with IVD and Muscle
Embryonic Tissue Antibodies	>33 publications with IVD and Embryonic Tissue
Liver Antibodies	>28 publications with IVD and Liver