Dystrophin immunoreactivity was weak or totally absent in the aganglionic bowel (Figure 1, B; Table).
The lack of proteins from the 3 major domains of the cytoskeleton and membrane skeleton in the aganglionic bowel suggests an altered cytoskeleton in the contracted bowel segment.
An important unanswered question remains: what is the relationship between the hypertrophic nerve trunks and the structurally and functionally abnormal smooth muscle cells in the aganglionic bowel?
Smooth muscle from aganglionic bowel in Hirschsprung's disease impairs neuronal development in vitro.
10 Rectosigmoid resections Aganglionic Submucosal 10 .
A similar pattern of staining was seen in hypertrophic nerves in the myenteric plexus of aganglionic colon (Figure 2, A).
In 10 cases of typical rectosigmoid HD, abundant GLUT-1-positive fibers were present in the submucosal and myenteric plexuses of the aganglionic segments, and numerous fibers greater than 50 [micro]m were readily found in each (Figure 5, A).
In aganglionic tissue, hypertrophic nerve fibers in the myenteric and submucosal zones stained strongly, and in the larger fibers a distinct perineurium, similar to that seen in the serosal nerves in healthy colon, also stained (Figure 2, B).
In the aganglionic bowel (Figure 2) all the large hypertrophic fibers were NGFR positive and showed a distinct perineurium, but in smaller nerve fibers a distinct perineurium could not be identified.