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QARS1

Function

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

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.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the class-I aminoacyl-tRNA synthetase family.

Tissue Specificity

Highly expressed in fetal cerebral cortex, particularly in the ventricular zone, inner subventricular zone, outer subventricular zone, and cortical plate.

Cellular localization

Alternative names

QARS, QARS1, Glutamine--tRNA ligase, Glutaminyl-tRNA synthetase, GlnRS

swissprot:P47897 entrezGene:5859 omim:603727