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Aspartyl-tRNA Synthetase 2, Mitochondrial (DARS2) (C-Term) Peptide
|Synonyms||LBSL, ASPRS, FLJ10514, MT-ASPRS, RP3-383J4.2, MGC157196, si:dkey-21n10.1, DKFZp469I0720|
|2 references available|
|Price||49.50 $ Plus shipping costs $45.00|
|Availability||Will be delivered in 2 to 3 Business Days|
|Characteristics||Blocking peptide for DARS2 (C-term) antibody ABIN392298|
|Specificity||The synthetic peptide sequence used to generate the antibody AP7836b was selected from the C-term region of humanRS2. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
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) [MIM:611105]. 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.
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles|
|Restrictions||For Research Use only|
Bonnefond, Fender, Rudinger-Thirion et al.: "Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS." in: Biochemistry, Vol. 44, Issue 12, pp. 4805-16, 2005 (PubMed).
Scheper, van der Klok, van Andel et al.: "Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation." in: Nature genetics, Vol. 39, Issue 4, pp. 534-9, 2007 (PubMed).