Anti-QRSL1 Antibody Picoband® HRP Conjugated

Contributes to glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity. Involved in glutaminyl-tRNAGln biosynthesis via transamidation and mitochondrial translation. Located in mitochondrion. Part of glutamyl-tRNA (Gln) amidotransferase complex. Implicated in combined oxidative phosphorylation deficiency 40.

E.coli-derived human QRSL1 recombinant protein (Position: M1-Q528) . Human QRSL1 shares 88.1% and 87% amino acid (aa) sequence identity with mouse and rat QRSL1, respectively.

1. Friederich, M. W., Timal, S., Powell, C. A., Dallabona, C., Kurolap, A., Palacios-Zambrano, S., Bratkovic, D., Derks, T. G. J., Bick, D., Bouman, K., Chatfield, K. C., Damouny-Naoum, N., and 41 others. Pathogenic variants in glutamyl-tRNA (Gln) amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder. Nature Commun. 9: 4065, 2018. Note: Electronic Article. 2. Hartz, P. A. Personal Communication. Baltimore, Md. 11/16/2016. 3. Kamps, R., Szklarczyk, R., Theunissen, T. E., Hellebrekers, D. M. E. I., Sallevelt, S. C. E. H., Boesten, I. B., de Koning, B., van den Bosch, B. J., Salomons, G. S., Simas-Mendes, M., Verdijk, R., Schoonderwoerd, K., de Coo, I. F. M., Vanoevelen, J. M., Smeets, H. J. M. Genetic defects in mtDNA-encoded protein translation cause pediatric, mitochondrial cardiomyopathy with early-onset brain disease. Europ. J. Hum. Genet. 26: 537-551, 2018.