A. Marbini et al., IMMUNOHISTOCHEMICAL LOCALIZATION OF UTROPHIN AND OTHER CYTOSKELETAL PROTEINS IN SKIN SMOOTH-MUSCLE IN NEUROMUSCULAR DISEASES, Journal of the neurological sciences, 143(1-2), 1996, pp. 156-160
We investigated the immunohistochemical distribution of cytoskeletal p
roteins in smooth muscles of 15 patients with Duchenne muscular dystro
phy (DMD), 8 patients with Becker muscular dystrophy (BMD), 28 patient
s with various neuromuscular diseases, and 2 normal controls, performi
ng skin and muscle biopsies. Dystrophin immunostaining confirmed absen
t reaction in thr arrector pill muscles of DMD patients, faint positiv
e reaction in BMD patients, and strong dystrophin reaction in patients
with other neuromuscular diseases and normal controls. Immunostaining
of utrophin was positive with variable intensity in the arrector pill
muscles in all DMD patients. In BMD patients, utrophin was faintly ex
pressed in the arrector pill muscles in 2 cases, and negative in the o
ther 5 patients. Zn the other cases of neuromuscular diseases and in n
ormal controls, immunostaining for utrophin was negative in the arrect
or pili muscles. Staining of the capillary endothelial cells and muscu
lar Vessel walls was seen in normal controls, as well as in DMD, BMD,
and other neuromuscular diseases. Vinculin, vimentin and desmin were e
xpressed both in arrector pill smooth muscles and in vessel walls of p
atients with dystrophinopathy and other neuromuscular diseases, as wel
l as in normal controls. Thus utrophin is normally expressed in the sm
ooth muscle of the vessels and its expression does not vary in neuromu
scular diseases. On the contrary, in the arrector pili smooth muscle u
trophin is not expressed in normal controls but it is in dystrophinopa
thies, paralleling the findings in striated muscle, which expresses ut
rophin in a reciprocal manner with respect to dystrophin.