Variants in different genes encoding mitochondrial ARSs lead to phenotypes that are specific to tissues.3Neurological disease is associated with variants of five ARS genes (IARS2,DARS2,RARS2,FARS2,EARS2).4, 5It is noteworthy that Isoleucyl-tRNA synthetase 2 (IARS2) gene is an important member of ARS family.IARS2gene, located at 1q41, spans approximately 53.9 kb and contains 23 exons. This evidence concerns the gene IARS2 and glycogen storage disease VI.