QARS

Aminoacyl-tRNA synthetases catalyze the aminoacylation of tRNA by their cognate amino acid. Because of their central role in linking amino acids with nucleotide triplets contained in tRNAs, aminoacyl-tRNA synthetases are thought to be among the first proteins that appeared in evolution. In metazoans, 9 aminoacyl-tRNA synthetases specific for glutamine (gln), glutamic acid (glu), and 7 other amino acids are associated within a multienzyme complex. Although present in eukaryotes, glutaminyl-tRNA synthetase (QARS) is absent from many prokaryotes, mitochondria, and chloroplasts, in which Gln-tRNA(Gln) is formed by transamidation of the misacylated Glu-tRNA(Gln). Glutaminyl-tRNA synthetase belongs to the class-I aminoacyl-tRNA synthetase family. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2013]

Full Name

Glutaminyl-TRNA Synthetase

Function

Glutamine--tRNA ligase (PubMed:26869582).
Plays a critical role in brain development (PubMed:24656866).

Biological Process

Biological Process brain developmentManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process glutaminyl-tRNA aminoacylationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process negative regulation of apoptotic signaling pathwayManual Assertion Based On ExperimentIDA:CAFA
Biological Process negative regulation of protein kinase activityManual Assertion Based On ExperimentIDA:CAFA
Biological Process negative regulation of stress-activated MAPK cascadeManual Assertion Based On ExperimentIDA:CAFA
Biological Process negative regulation of transcription, DNA-templatedManual Assertion Based On ExperimentIDA:CAFA
Biological Process tRNA aminoacylation for protein translationTAS:Reactome

Cellular Location

Cytoplasm, cytosol
Cytoplasm

Involvement in disease

Microcephaly, progressive, with seizures and cerebral and cerebellar atrophy (MSCCA):
A severe, autosomal recessive, neurodevelopmental and neurodegenerative disorder characterized by progressive microcephaly, severe seizures in infancy, atrophy of the cerebral cortex and cerebellar vermis, and mild atrophy of the cerebellar hemispheres, resulting in profoundly delayed development and hypotonia.