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>   首页   >   产品   >   一抗   >   其他   >   L2HGDH Rabbit pAb   

L2HGDH Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF
Primary Accession Q9H9P8
Other Accession Q9H9P8
Host Rabbit
Clonality Polyclonal
Calculated MW 50316 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human L2HGDH
Epitope Specificity 201-300/463
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the L2HGDH family.
DISEASE Defects in L2HGDH are the cause of L-2-hydroxyglutaric aciduria (L2HGA) [MIM:236792]. 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions 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]
Additional Information
Gene ID 79944
Other Names C14orf160; L2HGA; RGD1306250; L2HDH_BOVIN; L2HGDH; Duranin; 1.1.99.2; L2HDH_HUMAN; L2HDH_MOUSE; L2HDH_PONAB; L-2-hydroxyglutarate dehydrogenase; chromosome 14 open reading frame 160; 2-hydroxyglutarate dehydrogenase
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name L2HGDH (HGNC:20499)
Function Catalyzes the oxidation of L-2-hydroxyglutarate to 2- oxoglutarate (alpha-ketoglutarate).
Cellular Location Mitochondrion.
Tissue Location 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.
Research Areas
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