Recombinant protein encompassing a sequence within the center region of human AspRS. The exact sequence is proprietary.
Alternative Synonym:
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).
