Our study shows that: (i) hAAT has a positive effect on compound muscle potential in CMT1A mice, indicating partial recovery of axonal degeneration; (ii) hAAT can ameliorate histopathological signs of CMT1A; (iii) hAAT can reduce long-term inflammatory response in vivo (plasma IL-6); and (iv) hAAT mode action can be driven by different molecular mechanisms related to ADAM-17 inhibition, MHCII repression, and inflammatory response modulation. This evidence concerns the gene IL6 and Charcot-Marie-Tooth disease type 1A.