We studied the immunolocalization of Dp116 (a 116 kDa protein product
of the dystrophin gene), vinculin, talin, vimentin, desmin, spectrin a
nd titin in the sural nerve biopsies of 25 patients with peripheral ne
uropathies of different origin. 4 patients presented with HMSN type 1,
4 with HMSN type 2, 2 with HNPP, 4 with CIDP, 5 with chronic axonal n
europathy of unknown origin, 3 with vasculitic neuropathy, 3 with diab
etic neuropathy. Expression and localization of Dp116, vinculin, vimen
tin, desmin, spectrin and titin did not differ from normal control cas
es. Spectrin and titin immunoreactivities were absent and desmin was o
ccasionally found in few epineurial vessels. A thin rim of Dp116 bindi
ng surrounded the outermost layer of myelin sheaths. Perineurium and e
pineurial vessels stained deeply for vinculin. Vimentin immunoreactivi
ty was seen in all endoneurial, perineurial and epineurial cells. Immu
noreactivity for talin was normally found at endoneurial and epineuria
l vessel walls, perineurial cells and epineurial fibroblasts in all th
e sural nerves except diabetic nerves. In the latter, whereas talin bi
nding was normal in the vessel walls and epineurial fibroblasts, it wa
s markedly reduced in the perineurium. On immunoblot, two bands at 235
and 190 kDa were found in the sural nerves with the antibody anti-tal
in, and both were reduced only in the patients with diabetic neuropath
y. We postulate that decreased perineurium talin in diabetic polyneuro
pathy may be related to the known alterations of the tight junctions o
f the perineurial cells, which have been proposed to be a contributory
factor to impaired permeability barrier properties. (C) 1997 Elsevier
Science B.V.