A transgenic mouse generated with the MFN2 point mutation MFN2R94Q, presents neurologic features seen in CMT2A patients, such as severe early-onset sensorimotor deficits, vision loss, and axonal degeneration, and it was shown that increased expression of MFN1 in the nervous system rescued the MFN2 mutant phenotypes, highlighting the importance of MFN isoforms balance and providing a potential therapeutic strategy for this disease [214]. Here, MFN2 is linked to Charcot-Marie-Tooth disease type 2A1.