Rabbit Anti-L2HGDH Polyclonal Antibody – Cat# MBS6315014

L2HGDH Antibody – Rabbit Polyclonal

Name: L2HGDH Antibody; Polyclonal; Rabbit; Human/Mouse-Reactive; MyBioSource
Brand: MyBioSource
SKU: MBS6315014
Price: 1060 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6315014

This gene encodes L-2-hydroxyglutarate dehydrogenase, a FAD-dependent enzyme that oxidizes L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene cause L-2-hydroxyglutaric aciduria, a rare autosomal recessive… More details

$1060 / 0.1 mL +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

L2HGDH, Polyclonal Antibody

Full Product Name

L2HGDH, ID (L2HGDH, C14orf160, L-2-hydroxyglutarate dehydrogenase, mitochondrial, Duranin) (MaxLight 490)

Product Gene Name

anti-L2HGDH antibody

Product Synonym Names

L2HGDH; C14orf160; L-2-hydroxyglutarate dehydrogenase, mitochondrial; Duranin

Clonality

Polyclonal

Isotype

IgG

Host

Rabbit

Species Reactivity

Human, Mouse

Form/Format

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight490.

Purity/Purification

Purified by Protein A Affinity Chromatography.

Preparation and Storage

Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight490 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Molecular Weight

50,316 Da

Chromosome Location

Chromosome: 14; NC_000014.8 (50704285..50778947, complement). Location: 14q21.3

OMIM

236792

3D Structure

ModBase 3D Structure for Q9H9P8

Immunogen

L2HGDH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 139-167 amino acids from the Central region of human L2HGDH.

Conjugate

MaxLight490

Note

Preservative Free

Product Categories/Family for anti-L2HGDH antibody

Antibodies; Mitochondrial Proteins

Applications Tested/Suitable for anti-L2HGDH antibody

Western Blot (WB), FLISA

Application Notes for anti-L2HGDH antibody

WB: 1:100-500
FLISA: 1:1,000
Applications are based on unconjugated antibody.

Product References and Citations for anti-L2HGDH antibody

Short, A.D., et al. Vet. Rec. 167(12):455-457(2010). Steenweg, M.E., et al. Hum. Mutat. 31(4):380-390(2010). Kranendijk, M., et al. Hum. Mutat. 31(3):279-283(2010). Vilarinho, L., et al. J. Hum. Genet. 55(1):55-58(2010). Haliloglu, G., et al. Neuropediatrics 39(2):119-122(2008).

Other Notes

Small volumes of anti-L2HGDH 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 L2HGDH purchase

MBS6315014 is an antibody targeting L2HGDH. Also known as L2HGDH, ID (L2HGDH, C14orf160, L-2-hydroxyglutarate dehydrogenase, mitochondrial, Duranin) (MaxLight 490). Reactive in Human, Mouse. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for L2HGDH. It may not necessarily be applicable to this product.

NCBI GI #

13376331

NCBI GeneID

79944

NCBI Accession #

NP_079160.1

UniProt Primary Accession #

Q9H9P8

UniProt Secondary Accession #

Q9BRR1

UniProt Related Accession #

Q9H9P8

GenBank Nucleotide #

NM_024884.2

NCBI Official Full Name

L-2-hydroxyglutarate dehydrogenase, mitochondrial

NCBI Official Synonym Full Names

L-2-hydroxyglutarate dehydrogenase

NCBI Official Symbol

L2HGDH

NCBI Official Synonym Symbols

C14orf160

UniProt Protein Name

L-2-hydroxyglutarate dehydrogenase, mitochondrial

UniProt Synonym Protein Names

Duranin

UniProt Gene Name

L2HGDH

UniProt Synonym Gene Names

C14orf160

UniProt Entry Name

L2HDH_HUMAN

NCBI Protein Information

L-2-hydroxyglutarate dehydrogenase, mitochondrial; duranin; alpha-ketoglutarate reductase; alpha-hydroxyglutarate oxidoreductase; L-alpha-hydroxyglutarate dehydrogenase

UniProt Comments for L2HGDH

Catalytic activity: (S)-2-hydroxyglutarate + acceptor = 2-oxoglutarate + reduced acceptor. Ref.5

Cofactor: FAD. Ref.5

Subcellular location: Mitochondrion Ref.5.

Tissue specificity: Widely expressed. Highly expressed in brain, testis and muscle. Expressed to a lower extent in lymphocytes, fibroblasts, keratinocytes, placenta, bladder, small intestine, liver and bone marrow. Ref.8

Involvement in disease: L-2-hydroxyglutaric aciduria (L2HGA) [MIM:236792]: A rare autosomal recessive disorder clinically characterized by mild psychomotor delay in the first years of life, followed by progressive cerebellar ataxia, dysarthria and moderate to severe mental retardation. Diagnosis is based on the presence of an excess of L-2-hydroxyglutaric acid in urine, blood and cerebrospinal fluid.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.8 Ref.9

Miscellaneous: Was named 'duranin' in honor of Marinus Duran, who first described L-2-hydroxyglutaric aciduria.

Sequence similarities: Belongs to the L2HGDH family.

Biophysicochemical propertiesKinetic parameters:KM=800 µM for L-2-hydroxyglutarate Ref.5

Research Articles on L2HGDH

1. Report ten novel mutations in the L2HGDH gene in patients with L-2-hydroxyglutaric aciduria from different ethnic backgrounds.

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 anti-L2HGDH antibody

Reference Product	PubMed Publications
D2HGDH antibody	>2 publications with L2HGDH and D2HGDH

Pathways associated with anti-L2HGDH antibody

Products by Pathway	Pathway Diagram
Butanoate Metabolism Pathway antibodies	Butanoate Metabolism Pathway Diagram
Butanoate Metabolism Pathway antibodies	Butanoate Metabolism Pathway Diagram
Interconversion Of 2-oxoglutarate And 2-hydroxyglutarate Pathway antibodies	Interconversion Of 2-oxoglutarate And 2-hydroxyglutarate Pathway Diagram
Metabolism Pathway antibodies	Metabolism Pathway Diagram
Pyruvate Metabolism And Citric Acid (TCA) Cycle Pathway antibodies	Pyruvate Metabolism And Citric Acid (TCA) Cycle Pathway Diagram
The Citric Acid (TCA) Cycle And Respiratory Electron Transport Pathway antibodies	The Citric Acid (TCA) Cycle And Respiratory Electron Transport Pathway Diagram

Diseases associated with anti-L2HGDH antibody

Disease Name	Pubmed Publications
L-2-HYDROXYGLUTARIC ACIDURIA Antibodies	>20 publications with L2HGDH and L-2-HYDROXYGLUTARIC ACIDURIA
Nervous System Diseases Antibodies	>18 publications with L2HGDH and Nervous System Diseases
Brain Diseases Antibodies	>18 publications with L2HGDH and Brain Diseases
Seizures Antibodies	>4 publications with L2HGDH and Seizures
Disease Models, Animal Antibodies	>2 publications with L2HGDH and Disease Models, Animal
Cognition Disorders Antibodies	>1 publications with L2HGDH and Cognition Disorders

Organs/Tissues associated with anti-L2HGDH antibody

Organ/Tissue Name	Pubmed Publications
Brain Antibodies	>18 publications with L2HGDH and Brain
Blood Antibodies	>5 publications with L2HGDH and Blood
Liver Antibodies	>1 publications with L2HGDH and Liver