Aspartyl-tRNA Synthetase 2, Mitochondrial (DARS2) (C-Term) antibody

Antigen: Aspartyl-tRNA Synthetase 2, Mitochondrial (DARS2)

Synonyms: LBSL, ASPRS, FLJ10514, MT-ASPRS, RP3-383J4.2, MGC157196, si:dkey-21n10.1, DKFZp469I0720

Binding Site: C-Term

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Application: Western Blotting (WB), Immunohistochemistry (IHC), ELISA

Certificates: ISO 9001:2008

Catalog no.: ABIN392298

Additional Information

Alternative name: DARS2

Gene ID: 55157

UniProt: Q6PI48

Immunogen: This DARS2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 616~645 amino acids from the C-terminal region of human DARS2.

Isotype: Ig

Description: Other names: Aspartyl-tRNA synthetase, mitochondrial [Precursor], EC 6.1.1.12, Aspartate--tRNA ligase, AspRS, DARS2

Characteristics: Purified Rabbit Polyclonal Antibody (Pab)

Specificity: This DARS2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 616~645 amino acids from the C-terminal region of human DARS2.

Molecular Weight: 73563 DA

Comments:

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.

Application Details

Application Notes: The suggested dilution is: ELISA ~~ 1:1,000 Western blotting~~ 1:50~100 Immunohistochemistry ~~ 1:10~50

Concentration: 0.25 mg/ml

Purification: Purified

Buffer: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Storage: Maintain refrigerated at 2-8 deg C for up to 6 months. For long term storage store at -20 deg C in small aliquots to prevent freeze-thaw cycles

Research Area: Translation Factors, Signaling, Cell Structure

Restrictions: For Research Use only

Images

Western blot analysis of anti-DARS2 Antibody (C-term) (ABIN392298) in K562 cell line lysates (35ug/lane). DARS2(arrow) was detected using the purified Pab (1:60 dilution).

Formalin-fixed and paraffin-embedded human skeletal muscle tissue reacted with DARS2 antibody (C-term) (ABIN392298), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry. clinical relevance has not been evaluated.

Publications

Product: 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).