Full Product Name
DARS2 (Aspartyl-tRNA Synthetase, Mitochondrial, Aspartate-tRNA Ligase, AspRS)
Product Synonym Names
Anti -DARS2 (Aspartyl-tRNA Synthetase, Mitochondrial, Aspartate-tRNA Ligase, AspRS)
Product Gene Name
anti-DARS2 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence
MYFPSWLSQL YRGLSRPIRR TTQPIWGSLY RSLLQSSQRR IPEFSSFVVR TNTCGELRSS HLGQEVTLCG WIQYRRQNTF LVLRDFDGLV QVIIPQDESA ASVKKILCEA PVESVVQVSG TVISRPAGQE NPKMPTGEIE IKVKTAELLN ACKKLPFEIK NFVKKTEALR LQYRYLDLRS FQMQYNLRLR SQMVMKMREY LCNLHGFVDI ETPTLFKRTP GGAKEFLVPS REPGKFYSLP QSPQQFKQLL MVGGLDRYFQ VARCYRDEGS RPDRQPEFTQ IDIEMSFVDQ TGIQSLIEGL LQYSWPNDKD PVVVPFPTMT FAEVLATYGT DKPDTRFGMK IIDISDVFRN TEIGFLQDAL SKPHGTVKAI CIPEGAKYLK RKDIESIRNF AADHFNQEIL PVFLNANRNW NSPVANFIME SQRLELIRLM ETQEEDVVLL TAGEHNKACS LLGKLRLECA DLLETRGVVL RDPTLFSFLW VVDFPLFLPK EENPRELESA HHPFTAPHPS DIHLLYTEPK KARSQHYDLV LNGNEIGGGS IRIHNAELQR YILATLLKED VKMLSHLLQA LDYGAPPHGG IALGLDRLIC LVTGSPSIRD VIAFPKSFRG HDLMSNTPDS VPPEELKPYH IRVSKPTDSK AERAH
Chromosome Location
Chromosome: 1; NC_000001.10 (173793719..173827682). Location: 1q25.1
3D Structure
ModBase 3D Structure for Q6PI48
Specificity
Recognizes human DARS2.
Purity/Purification
Affinity Purified
Purified by Protein A affinity chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2.
Immunogen
Full length human DARS2, aa1-645 (NP_060592.2).
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
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.
Related Product Information for
anti-DARS2 antibody
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.
Product Categories/Family for anti-DARS2 antibody
Antibodies; Abs to Enzymes, Synthase
Applications Tested/Suitable for anti-DARS2 antibody
Western Blot (WB)
Application Notes for anti-DARS2 antibody
Suitable for use in Western Blot.
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 Primary Accession #
Q6PI48
[Other Products]
UniProt Related Accession #
Q6PI48[Other Products]
Molecular Weight
73,563 Da[Similar Products]
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; RP3-383J4.2
??[Similar Products]
NCBI Protein Information
aspartate--tRNA ligase, mitochondrial; aspartate tRNA ligase 2, mitochondrial; aspartyl-tRNA synthetase, mitochondrial
UniProt Protein Name
Aspartate--tRNA ligase, mitochondrial
UniProt Synonym Protein Names
Aspartyl-tRNA synthetase
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; Translation; Mitochondrial; Ligase
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
Research Articles on DARS2
1. A comparison of biophysical properties of human mitochondrial aspartyl-tRNA synthetase, HsaDRS2, with them to those of a bacterial (E. coli) homolog, EcoDRS.
Precautions
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Disclaimer
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