Hereditary neuropathies are classified into several subtypes according to 
clinical, electrophysiologic and pathologic findings. Recent genetic studies 
have revealed their phenotypic and genetic diversities. In the primary 
peripheral demyelinating neuropathies (CMT1), at least 15 genes have been 
associated with the disorders; altered dosage or point mutation of PMP22, GJB1, 
MPZ, EGR2, MTMR2, NDRG1, PRX, SOX10, GDAP1 and MTMR13/SBF2. In the primary 
peripheral axonal neuropathies (CMT2), at least 10 genes have been associated 
with these disorders; NEFL, KIF1B, MFN2, GAN1, LMNA, RAB7, GARS, TDP1, APTX, and 
SETX. In addition, some mutations in GJB1, MPZ, GDAP1 and NEFL also present with 
clinical and electrophysiologic findings of CMT2. Patients with TDP1, APTX or 
SETX mutations share common clinical findings; autosomal recessive inheritance, 
cerebellar ataxia, and axonal neuropathy. These genes are suspected to be 
related to DNA/RNA repair and induce cell death especially in neuronal cells. In 
addition to the above diseases, we have reported a new type of NMSNP (MIM# * 
604484) characterized by proximal dominant neurogenic atrophy, obvious sensory 
nerve involvement and the gene locus on 3q12.3. Here, we summarize the genetic 
bases of hereditary neuropathies and attempt to highlight significant 
genotype-phenotype correlations with a special interest in nonsense-mediated 
mRNA decay pathway.