Mm. Portier et al., PERIPHERIN AND NEUROFILAMENTS - EXPRESSION AND ROLE DURING NEURAL DEVELOPMENT, Comptes rendus de l'Academie des sciences. Serie 3, Sciences de la vie, 316(9), 1993, pp. 1133-1140
This review focuses attention on the expression of peripherin and the
low-molecular mass neurofilament protein during development, as well a
s on recent results concerning the roles of these neuronal proteins. P
eripherin is the only type III intermediate filament that has been sho
wn to be expressed in neurons but exclusively in motor, sensory and sy
mpathetic neurons ; moreover, it is co-expressed with neurofilament pr
oteins (NFP). Clearly, peripherin is expressed concomitantly with axon
al growth during development, and its synthesis appears necessary to a
xonal regeneration in the adult. As to NFP, they are presumed to maint
ain the axonal diameter and thereby ensure a normal conduction velocit
y. In many neuropathies, either occurring in man or provoked by differ
ent means in animals, the neurofilament network is disrupted thus givi
ng rise to bundles of filaments in perikarya or along axons ; conseque
ntly, the axonal transport is impaired. The possible significance of t
he overexpression of NFP is discussed.