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anti-L2HGDH antibody :: Rabbit L-2-hydroxyglutarate dehydrogenase Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS714368 anti-L2HGDH antibody
Unit / Price
0.05 mL  /  $230 +1 FREE 8GB USB
0.15 mL  /  $500 +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   

L-2-hydroxyglutarate dehydrogenase (L2HGDH), Polyclonal Antibody

 Also Known As   

Rabbit anti-human L-2-hydroxyglutarate dehydrogenase polyclonal Antibody

 Product Synonym Names    L-2-hydroxyglutarate dehydrogenase; L2HGDH; C14orf160; DURANIN; FLJ12618
 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    609584
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Antigen Affinity Purified
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 Immunogen    Human L2HGDH
 Storage Buffer    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
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 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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 Applications Tested/Suitable for anti-L2HGDH antibody   

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

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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 #    119586129
 NCBI GeneID    79944
 NCBI Accession #    EAW65725.1 [Other Products]
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 UniProt Secondary Accession #    Q9BRR1 [Other Products]
 UniProt Related Accession #    Q9H9P8 [Other Products]
 Molecular Weight    48,505 Da [Similar Products]
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 NCBI Official Full Name    L-2-hydroxyglutarate dehydrogenase
 NCBI Official Synonym Full Names    L-2-hydroxyglutarate dehydrogenase
 NCBI Official Symbol    L2HGDH [Similar Products]
 NCBI Official Synonym Symbols   
C14orf160
[Similar Products]
 NCBI Protein Information    L-2-hydroxyglutarate dehydrogenase, mitochondrial; duranin; alpha-ketoglutarate reductase; alpha-hydroxyglutarate oxidoreductase; L-alpha-hydroxyglutarate dehydrogenase
 UniProt Protein Name    L-2-hydroxyglutarate dehydrogenase, mitochondrial
 UniProt Synonym Protein Names   
Duranin
 Protein Family    L-2-hydroxyglutarate dehydrogenase
 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: Oxidoreductase; EC 1.1.99.2; Mitochondrial; Carbohydrate Metabolism - butanoate

Chromosomal Location of Human Ortholog: 14q21.3

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

Molecular Function: 2-hydroxyglutarate dehydrogenase activity

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

Disease: L-2-hydroxyglutaric Aciduria
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 Research Articles on L2HGDH    1. modest increases in intracellular 2-HG in acute myeloid leukaemia cells, as seen with the rs11554137 SNP, might enhance chemoresistance, or promote acquisition of leukaemia-iniating mutations
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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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Products associated with anti-L2HGDH antibodyPathways associated with anti-L2HGDH antibody
 Reference Product  PubMed Publications
 D2HGDH antibody  >2 publications with L2HGDH and D2HGDH
 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 antibodyOrgans/Tissues associated with anti-L2HGDH antibody
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >19 publications with L2HGDH and Nervous System Diseases
 Brain Diseases Antibodies  >19 publications with L2HGDH and Brain Diseases
 Epilepsy Antibodies  >7 publications with L2HGDH and Epilepsy
 2-Hydroxyglutaricaciduria Antibodies  >6 publications with L2HGDH and 2-Hydroxyglutaricaciduria
 Mental Disorders Antibodies  >4 publications with L2HGDH and Mental Disorders
 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
 Intellectual Disability Antibodies  >1 publications with L2HGDH and Intellectual Disability
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >20 publications with L2HGDH and Brain
 Blood Antibodies  >6 publications with L2HGDH and Blood
 Liver Antibodies  >1 publications with L2HGDH and Liver
 Kidney Antibodies  >1 publications with L2HGDH and Kidney
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