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L2HGDH Antibody (Center)

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - L2HGDH Antibody (Center) AP10255c
    L2HGDH Antibody (Center) (Cat. #AP10255c) western blot analysis in MDA-MB435 cell line and mouse kidney tissue lysates (35ug/lane).This demonstrates the L2HGDH antibody detected the L2HGDH protein (arrow).
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Product info
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, E
Primary Accession Q9H9P8
Other Accession NP_079160.1
Reactivity Human, Mouse
Host Rabbit
Clonality Polyclonal
Isotype Rabbit Ig
Calculated MW 50316 Da
Additional info
Gene ID 79944
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.
Dilution WB~~1:1000
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.
StorageMaintain 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.
PrecautionsL2HGDH Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name L2HGDH
Synonyms C14orf160
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

BACKGROUND

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.

REFERENCES

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)

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