Produced in rabbits immunized with E. coli-derived Human LARS2 fragment, and purified by antigen affinity chromatography.
Source
Polyclonal Rabbit IgG
Purification
Protein A & Antigen Affinity
Formulation
PBS, pH7.0 with 0.03% Proclin300
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Storage
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Avoid repeated freeze-thaw cycles.
LARS2 is located in the chromosome 3 common eliminated region-1 (C3CER1) at 3p21.3 and encodes mitochondrial leucyl-tRNA synthetase (mtLeuRS), a 903 amino acid protein. This enzyme is important in the production (synthesis) of proteins in cellular structures called mitochondria, the energy-producing centers in cells. Aminoacyl-tRNA synthetases then attach specific amino acids to the 3- ends of their cognate tRNAs, which is required in the cytoplasm and mitochondria for the translation of nuclear and mitochondrial encoded genes, respectively. Apart from two aminoacyl transfer RNA synthases, glycyl-tRNA synthetase and lysyl-tRNA synthetase, all other synthetases are encoded by separate genes for nuclear and mitochondrial functions.
References
Riley LG, et al. (2016) Lars2 variants associated with hydrops, lactic acidosis, sideroblastic anemia, and multisystem failure. JIMD Rep 28 49-57.