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

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Catalog # MBS7046661
Unit / Price
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  0.05 mL  /  $120 +1 FREE 8GB USB
  0.1 mL  /  $160 +1 FREE 8GB USB
Product Name

L2HGDH, Polyclonal Antibody

Popular Item
Full Product Name

L2HGDH Antibody

Product Synonym Names
L-2-hydroxyglutarate dehydrogenase, mitochondrial; Duranin; L2HGDH; C14orf160
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
236792
3D Structure
ModBase 3D Structure for Q9H9P8
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse
Purity/Purification
Antigen Affinity Purified
Form/Format
Liquid
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.
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 13485:2003 and EN ISO 13485:2012 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.
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)

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
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
NCBI/Uniprot data below describe general gene information for L2HGDH. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
48,505 Da
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
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]
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
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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