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DARS2 Antibody (C-term) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession Q6PI48
Clone Names 71228110
Additional Information
Gene ID 55157
Other Names Aspartate--tRNA ligase, mitochondrial, Aspartyl-tRNA synthetase, AspRS, DARS2
Target/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.
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name DARS2
Function Catalyzes the attachment of aspartate to tRNA(Asp) in a two- step reaction: aspartate is first activated by ATP to form Asp-AMP and then transferred to the acceptor end of tRNA(Asp).
Cellular Location Mitochondrion matrix Mitochondrion membrane
Research Areas
Citations (0)
citation

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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.

References

Scheper,G.C., Nat. Genet. 39 (4), 534-539 (2007)Bonnefond,L., Biochemistry 44 (12), 4805-4816 (2005)

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$ 277.78
Cat# BP7836b
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