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anti-L2HGDH antibody :: Rabbit anti-Human, Mouse L2HGDH Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS7046661
Unit / Price
0.05 mL  /  $120 +1 FREE 8GB USB
0.1 mL  /  $160 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

L2HGDH, Polyclonal Antibody

★Popular Item★
 Also Known As   

L2HGDH Antibody

 Product Synonym Names    L-2-hydroxyglutarate dehydrogenase, mitochondrial; Duranin; L2HGDH; C14orf160
 Product Gene Name   

anti-L2HGDH antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    236792
 3D Structure    ModBase 3D Structure for Q9H9P8
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse
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 Purity/Purification    Antigen Affinity Purified
 Form/Format    Liquid
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 Species    Human
 Immunogen    Recombinant human L-2-hydroxyglutarate dehydrogenase, mitochondrial protein (N-220AA)
 Conjugate    Non-conjugated
 Buffer    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 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.
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Related Product Information for anti-L2HGDH antibody

   integral component of membrane, integral component of mitochondrial inner membrane, mitochondrial inner membrane, mitochondrion, 2-hydroxyglutarate dehydrogenase activity, 2-oxoglutarate metabolic process, cellular protein metabolic process
 Applications Tested/Suitable for anti-L2HGDH antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

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 Western Blot (WB) of anti-L2HGDH antibody    Western blot
All lanes: L2HGDH antibody at 4.43 ug/ml
+Mouse heart tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 51,49 kDa
Observed band size: 50 kDa

anti-L2HGDH antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-L2HGDH antibody    Immunohistochemistry of paraffin-embedded human testis tissue using MBS7046661 at dilution 1:100
anti-L2HGDH antibody Immunohistochemistry (IHC) (IHC) image
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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 [Other Products]
 NCBI GenBank Nucleotide #    NM_024884.2 [Other Products]
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 UniProt Primary Accession #    Q9H9P8 [Other Products]
 UniProt Secondary Accession #    Q9BRR1 [Other Products]
 UniProt Related Accession #    Q9H9P8 [Other Products]
 Molecular Weight    48,505 Da
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 NCBI Official Full Name    L-2-hydroxyglutarate dehydrogenase, mitochondrial
 NCBI Official Synonym Full Names    L-2-hydroxyglutarate dehydrogenase
 NCBI Official Symbol    L2HGDH [Similar Products]
 NCBI Official Synonym Symbols   
L2HGA; C14orf160
[Similar Products]
 NCBI Protein Information    L-2-hydroxyglutarate dehydrogenase, mitochondrial
 UniProt Protein Name    L-2-hydroxyglutarate dehydrogenase, mitochondrial
 UniProt Synonym Protein Names   
Duranin
 UniProt Gene Name    L2HGDH [Similar Products]
 UniProt Synonym Gene Names    C14orf160 [Similar Products]
 UniProt Entry Name    L2HDH_HUMAN
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 NCBI Summary for L2HGDH    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 neurometabolic disorder resulting in moderate to severe mental retardation. [provided by RefSeq, Jul 2008]
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 UniProt Comments for L2HGDH    L2HGDH: Defects in L2HGDH are the cause of L-2-hydroxyglutaric aciduria (L2HGA). L2HGA is 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. Belongs to the L2HGDH family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.1.99.2; Oxidoreductase; Mitochondrial; Carbohydrate Metabolism - butanoate

Chromosomal Location of Human Ortholog: 14q21.3

Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrion

Molecular Function: 2-hydroxyglutarate dehydrogenase activity

Biological Process: 2-oxoglutarate metabolic process; cellular protein metabolic process

Disease: L-2-hydroxyglutaric Aciduria
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 Research Articles on L2HGDH    1. c.845G>A (p.R282Q) in the exon 7 of the L2HGDH gene is associated with 2-hydroxyglutaric aciduria in Chinese family.
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 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.
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Pathways associated with anti-L2HGDH antibodyDiseases 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
 Disease Name  Pubmed Publications
 Brain Diseases Antibodies  >24 publications with L2HGDH and Brain Diseases
 2-Hydroxyglutaricaciduria Antibodies  >11 publications with L2HGDH and 2-Hydroxyglutaricaciduria
 Epilepsy Antibodies  >8 publications with L2HGDH and Epilepsy
 Seizures Antibodies  >4 publications with L2HGDH and Seizures
 Disease Models, Animal Antibodies  >3 publications with L2HGDH and Disease Models, Animal
 Intellectual Disability Antibodies  >2 publications with L2HGDH and Intellectual Disability
 Kidney Diseases Antibodies  >1 publications with L2HGDH and Kidney Diseases
 Cognition Disorders Antibodies  >1 publications with L2HGDH and Cognition Disorders
Organs/Tissues associated with anti-L2HGDH antibody
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >25 publications with L2HGDH and Brain
 Blood Antibodies  >7 publications with L2HGDH and Blood
 Kidney Antibodies  >2 publications with L2HGDH and Kidney
 Liver Antibodies  >1 publications with L2HGDH and Liver
 Testis Antibodies  >1 publications with L2HGDH and Testis
 Embryonic Tissue Antibodies  >1 publications with L2HGDH and Embryonic Tissue
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