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

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

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

Certificates: ISO 9001:2008

Catalog no.: ABIN392299

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 257~287 amino acids from the center 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 257~287 amino acids from the center 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). 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 blot~~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: Metabolism, Cell Structure

Restrictions: For Research Use only

Images

Formalin-fixed and paraffin-embedded human testis tissue reacted with DARS2 antibody (Center) (ABIN392299), 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.

DARS2 Antibody (Center) (ABIN392299) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the DARS2 antibody detected the DARS2 protein (arrow).

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).