To our interest, two missense mutations of the BSCL2 gene, namely, N152S and S154L of Seipin, were found to dominantly cause distal hereditary motor neuropathy (dHMN) or distal muscular atrophy, which is characterized almost exclusively by the degeneration of motor nerve fibers, predominantly in the distal part of limbs (Windpassinger et al., 2004). The gene discussed is BSCL2; the disease is distal hereditary motor neuropathy.