|Application ||WB, E|
|Calculated MW||50316 Da|
|Other Names||L-2-hydroxyglutarate dehydrogenase, mitochondrial, Duranin, L2HGDH, C14orf160|
|Target/Specificity||This L2HGDH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 132-159 amino acids from the Central region of human L2HGDH.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||L2HGDH Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.|
|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.|
Provided below are standard protocols that you may find useful for product applications.
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.
Short, A.D., et al. Vet. Rec. 167(12):455-457(2010)
Steenweg, M.E., et al. Hum. Mutat. 31(4):380-390(2010)
Kranendijk, M., et al. Hum. Mutat. 31(3):279-283(2010)
Vilarinho, L., et al. J. Hum. Genet. 55(1):55-58(2010)
Haliloglu, G., et al. Neuropediatrics 39(2):119-122(2008)