Product Name
L2HGDH, Polyclonal Antibody
Popular Item
Full Product Name
L2HGDH Antibody
Product Synonym Names
L-2-hydroxyglutarate dehydrogenase; mitochondrial (EC:1.1.99.2); L2HGDH; C14orf160; Duranin
Product Gene Name
anti-L2HGDH antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9H9P8
Purity/Purification
Affinity Purified
Immunogen
Recombinant human L-2-hydroxyglutarate dehydrogenase, mitochondrial protein
Conjugation
Non-conjugated
Storage Buffer
Preservative: 0.03% Proclin 300; Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Preparation and Storage
Shipped at 4 degree C. Upon delivery, aliquot and store at -20 degree C or -80 degree C.
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 metabolic process, cellular protein metabolic process, small molecule metabolic process
Applications Tested/Suitable for anti-L2HGDH antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-L2HGDH antibody
Recommended dilution: WB:1:500-2000, IHC:1:20-1:200
Western Blot (WB) of anti-L2HGDH antibody
Western blot
All lanes: L2HGDH antibody at 8ug/ml+ K562 whole cell lysate
Goat polyclonal to rabbit at 1/10000 dilution
Predicted band size: 51,49 kDa
Observed band size: 50 kDa
Immunohistochemistry (IHC) of anti-L2HGDH antibody
Immunohistochemistry of paraffin-embedded human testis using MBS7002372 at dilution of 1:100
NCBI/Uniprot data below describe general gene information for L2HGDH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_079160.1
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NCBI GenBank Nucleotide #
NM_024884.2
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UniProt Primary Accession #
Q9H9P8
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UniProt Secondary Accession #
Q9BRR1[Other Products]
UniProt Related Accession #
Q9H9P8[Other Products]
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: Carbohydrate Metabolism - butanoate; Mitochondrial; EC 1.1.99.2; Oxidoreductase
Chromosomal Location of Human Ortholog: 14q21.3
Cellular Component: integral to membrane; integral to mitochondrial inner membrane; mitochondrial inner membrane; mitochondrion
Molecular Function: 2-hydroxyglutarate dehydrogenase activity
Biological Process: 2-oxoglutarate metabolic process; cellular metabolic process; cellular protein metabolic process
Disease: L-2-hydroxyglutaric Aciduria
Research Articles on L2HGDH
1. G mutation in the 14 studied cases.">Intragenic single nucleotide length polymorphisms and two extragenic microsatellites flanking the L2HGDH gene confirm the founder effect of c.241A>G mutation in the 14 studied cases.
Precautions
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Disclaimer
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