L2HGDH Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- 产品详情
- 实验流程
- 背景知识
Application ![]()
| WB, E |
---|---|
Primary Accession | Q9H9P8 |
Other Accession | NP_079160.1 |
Reactivity | Human, Mouse |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 50316 Da |
Antigen Region | 132-159 aa |
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 E~~Use at an assay dependent concentration. |
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. |
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. |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
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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