Anti-DARS2 Antibody Picoband® Fluoro647 Conjugated
Product Specifications
Background
DARS2 contains conserved residues involved in ATP binding, tRNA binding, and aspartic acid recognition, as well as catalytic site motifs characteristic of amino acid tRNA synthetases. 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) . The International Radiation Hybrid Mapping Consortium mapped the DARS2 gene to chromosome 1.
Synonyms
Aspartate--tRNA ligase, mitochondrial; Aspartyl-tRNA synthetase; AspRS; DARS2
Gene Name
DARS2
Gene ID
55157
UniProt
Q6PI48
Host
Rabbit
Reactivity
Human
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E.coli-derived human DARS2 recombinant protein (Position: D334-A448) .
Clonality
Polyclonal
Tissue Specificity
Detected in brain. Detected at very much lower levels in heart, lung, placenta and skeletal muscle. Highly expressed in cerebellum, but also found in frontal cortex, hippocampus and basal ganglia.
Applications
Flow Cytometry
Field of Research
DNA/RNA, Epigenetics and Nuclear Signaling, Metabolic Signaling Pathways, Metabolism, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Nucleotide Metabolism, Pathways and Processes, Signal Transduction, Translation
Purification
Immunogen affinity purified.
Form
Liquid
Function
Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD+ for the glycolytic pathway. Required for proper neuromuscular junction formation (Probable) .
References & Citations
1. Isohanni, P., Linnankivi, T., Buzkova, J., Lonnqvist, T., Pihko, H., Valanne, L., Tienari, P. J., Elovaara, I., Pirttila, T., Reunanen, M., Koivisto, K., Marjavaara, S., Suomalainen, A. DARS2 mutations in mitochondrial leucoencephalopathy and multiple sclerosis. J. Med. Genet. 47: 66-70, 2010. 2. Miyake, N., Yamashita, S., Kurosawa, K., Miyatake, S., Tsurusaki, Y., Doi, H., Saitsu, H., Matsumoto, N. A novel homozygous mutation of DARS2 may cause a severe LBSL variant. (Letter) Clin. Genet. 80: 293-296, 2011.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Calculated Molecular Weight
80420 MW
Specificity
No cross reactivity with other proteins.
Applications Notes
6
Gene Name Synonym
Aspartyl-tRNA synthetase 2, mitochondrial
Subcellular Location
Mitochondrion matrix.
Protein Name
Long-chain-fatty-acid--CoA ligase 3
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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