To allow for testing of allele-specific RNAi of mutant Gars1 as a potential treatment strategy for CMT2D, the Gars1delETAQ/+ mouse strain was crossed with mice homozygously carrying exon 8 of the human GARS1 gene (Gars1huEx8), producing compound heterozygous Gars1delETAQ/huEx8 mice. This evidence concerns the gene GARS1 and Charcot-Marie-Tooth disease type 2D.