Full Product Name
DARS2 Rabbit Polyclonal
Product Gene Name
anti-DARS2 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Aspartyl-tRNA synthetase 2, mitochondrial
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
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.
Applications Tested/Suitable for anti-DARS2 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for DARS2. It may not necessarily be applicable to this product.
NCBI Accession #
AAH45173.1
[Other Products]
UniProt Related Accession #
Q6PI48[Other Products]
Molecular Weight
73,563 Da
NCBI Official Full Name
DARS2 protein
NCBI Official Synonym Full Names
aspartyl-tRNA synthetase 2, mitochondrial
NCBI Official Symbol
DARS2 [Similar Products]
NCBI Official Synonym Symbols
LBSL; ASPRS; MT-ASPRS
[Similar Products]
NCBI Protein Information
aspartate--tRNA ligase, mitochondrial
UniProt Protein Name
Aspartate--tRNA ligase, mitochondrial
UniProt Synonym Protein Names
Aspartyl-tRNA synthetase; AspRS
UniProt Gene Name
DARS2 [Similar Products]
UniProt Synonym Gene Names
AspRS [Similar Products]
UniProt Entry Name
SYDM_HUMAN
NCBI Summary for DARS2
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]
UniProt Comments for DARS2
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: EC 6.1.1.12; Ligase; Translation; Mitochondrial
Chromosomal Location of Human Ortholog: 1q25.1
Cellular Component: mitochondrial matrix; mitochondrion; nucleus
Molecular Function: aspartate-tRNA ligase activity; aspartate-tRNA(Asn) ligase activity; ATP binding; protein binding; protein homodimerization activity; tRNA binding
Biological Process: tRNA aminoacylation; tRNA aminoacylation for protein translation
Disease: Leukoencephalopathy With Brainstem And Spinal Cord Involvement And Lactate Elevation
Research Articles on DARS2
1. This study identified DARS2-associated leukoencephalopathy with hypomyelination with brainstem and spinal cord involvement and leg spasticity.
Precautions
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