Except for PLEKHG5, many other causative genes have been reported in LMNDs: SMN1 and DYNC1H1in each autosomal recessive (AR) and dominant spinal muscular atrophy (SMA: MIM 253300 and 158600), HSPB1,HSPB8, and HSPB3in distal hereditary motor neuronopathy type 2 (dHMN2: MIM 608634, 158590 and 613376), GARS, BSCL2 and REEP1 in dHMN5 (MIM 600794 and 614751), IGHMBP2 in dHMN6 (MIM 604320), DCTN1 and SLC5A7 in dHMN7 (MIM 607641 and 158580), ATP7A in X-linked dHMN and SMA (MIM 300489), and SETX in dHMN with pyramidal sign (MIM 602433) [2-11]. Here, PLEKHG5 is linked to proximal spinal muscular atrophy.