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Official Full Name: aspartyl-tRNA synthetase 2, mitochondrial
Also known as: LBSL; ASPRS; mtAspRS; MT-ASPRS
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).