Histological features of neurogenic muscle involvement included type groupi
ng, muscle fiber atrophy, and target fibers. In muscles with myofiber atrop
hy and target fibers, we found an increased expression of the genes encodin
g for the ubiquitin-ATP-dependent proteolytic system. Thus, in patients wit
h target fibers, a 5.2- and a 3.9-fold increase were observed for the 2.4 a
nd 1.2 kb transcripts, respectively, while in those with atrophic angulated
hyperoxidative fibers, a 3.9- and a 4.4-fold increase were observed for th
e 2.4 and 1.2 kb transcripts, respectively. It is suggested that the activa
tion of this proteolytic system may be responsible for the skeletal muscle
alterations that often accompany human muscle neurogenic involvement. (C) 1
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