Peripheral nerves have peculiar energetic requirements because of considerable 
length of axons and therefore correct mitochondria functioning and distribution 
along nerves is fundamental. Mitochondrial dynamics refers to the continuous 
change in size, shape, and position of mitochondria within cells. Abnormalities 
of mitochondrial dynamics produced by mutations in proteins involved in 
mitochondrial fusion (mitofusin-2, MFN2), fission (ganglioside-induced 
differentiation-associated protein-1, GDAP1), and mitochondrial axonal transport 
usually present with a Charcot-Marie-Tooth disease (CMT) phenotype. MFN2 
mutations cause CMT type 2A by altering mitochondrial fusion and trafficking 
along the axonal microtubule system. CMT2A is an axonal autosomal dominant CMT 
type which in most cases is characterized by early onset and rather severe 
course. GDAP1 mutations also alter fission, fusion and transport of mitochondria 
and are associated either with recessive demyelinating (CMT4A) and axonal CMT 
(AR-CMT2K) and, less commonly, with dominant, milder, axonal CMT (CMT2K). OPA1 
(Optic Atrophy-1) is involved in fusion of mitochondrial inner membrane, and its 
heterozygous mutations lead to early-onset and progressive dominant optic 
atrophy which may be complicated by other neurological symptoms including 
peripheral neuropathy. Mutations in several proteins fundamental for the axonal 
transport or forming the axonal cytoskeleton result in peripheral neuropathy, 
i.e., CMT, distal hereditary motor neuropathy (dHMN) or hereditary sensory and 
autonomic neuropathy (HSAN), as well as in hereditary spastic paraplegia. 
Indeed, mitochondrial transport involves directly or indirectly components of 
the kinesin superfamily (KIF5A, KIF1A, KIF1B), responsible of anterograde 
transport, and of the dynein complex and related proteins (DYNC1H1, dynactin, 
dynamin-2), implicated in retrograde flow. Microtubules, neurofilaments, and 
chaperones such as heat shock proteins (HSPs) also have a fundamental role in 
mitochondrial transport and mutations in some of related encoding genes cause 
peripheral neuropathy (TUBB3, NEFL, HSPB1, HSPB8, HSPB3, DNAJB2). In this 
review, we address the abnormalities in mitochondrial dynamics and their role in 
determining CMT disease and related neuropathies.