LBSL; ASPRS; MT-ASPRS

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-DARS2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. It is a mitochondrial enzyme that specifically aminoacylates aspartyl-tRNA. Mutations in this gene are associated with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL).

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:200-1:2000
IHC: 1:50-1:200

Western blot analysis of extracts of various cell lines, using DARS2 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 90s.

645

aspartyl-tRNA synthetase 2, mitochondrial

aspartate--tRNA ligase, mitochondrial; aspartate tRNA ligase 2, mitochondrial; aspartyl-tRNA synthetase, mitochondrial

Aspartate--tRNA ligase, mitochondrial

AspRS??[Similar Products]

SYDM_HUMAN

The protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. It is a mitochondrial enzyme that specifically aminoacylates aspartyl-tRNA. Mutations in this gene are associated with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). [provided by RefSeq, Nov 2009]

DARS2: 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. Belongs to the class-II aminoacyl-tRNA synthetase family.

Protein type: Ligase; Translation; EC 6.1.1.12; Mitochondrial

Chromosomal Location of Human Ortholog: 1q25.1

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; nucleus

Molecular Function: protein binding; protein homodimerization activity; aspartate-tRNA ligase activity; aspartate-tRNA(Asn) ligase activity; ATP binding; tRNA binding

Biological Process: tRNA aminoacylation for protein translation; gene expression; tRNA aminoacylation

Disease: Leukoencephalopathy With Brainstem And Spinal Cord Involvement And Lactate Elevation

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