Synonyms | Aspartate-tRNA ligase, AspRS |
Swiss Prot Num | Q6PI48 |
Immunogen | This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the center region of human DARS2. |
Product Type | Antibodies |
Storage | Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch. |
References | 1. Scheper,G.C., Nat. Genet. 39 (4), 534-539 (2007) 2. Bonnefond,L., Biochemistry 44 (12), 4805-4816 (2005) |
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Format | State: Liquid purified Ig |
? | Purification: Prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS |
? | Buffer System: PBS with 0.09% (W/V) sodium azide |
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Specificity | This antibody reacts to DARS2. |
Application | ELISA: 1/1,000. Immunohistochemistry: 1/10 - 1/50. |
Background | Aspartyl-tRNA synthetase (DARS) is part of a multienzyme complex of aminoacyl-tRNA synthetases. Aspartyl-tRNA synthetase charges its cognate tRNA with aspartate during protein biosynthesis. Defects in DARS2 are a cause of leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). LBSL is an autosomal recessive disease and is defined on the basis of a highly characteristic constellation of abnormalities observed by magnetic resonance imaging and spectroscopy. Affected individuals develop slowly progressive cerebellar ataxia, spasticity, and dorsal column dysfunction, sometimes with a mild cognitive deficit or decline. |
Caution | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
Concentration | 0.25 mg/ml |