CHRNG and amyotrophic lateral sclerosis: In fact, while both mouse ALS strains present similar loss of MNs during the disease course [13], the rapid-progressor 129SvSOD1G93A mice present earlier axonal impairment and denervation of hind limb muscles as indicated by the marked upregulation of the AChRγ-subunit mRNA levels in the TA muscles compared to the C57SOD1G93A mice.