Abstract
A major obstacle to the understanding of pathophysiology of aganglionosis has been the lack of specific markers for various neuronal cell types that may be affected in this condition. Recently, with the availability of polyclonal and in particular monoclonal antibodies it has become possible to identify more precisely the innervation patterns in normal and aganglionic colon. We have produced a monoclonal antibody D which recognises a subset of axonal antigen which are distributed throughout the wall of normal rectum and colon.
Immunohistochemical scanning of the entire resected specimen of colon from 3 children with Hirschsprung's disease at 0.5 cm intervals, demonstrated large numbers of D7 immunoreactive nerve bundles in the circular muscle of the ganglionic colon, few fibres in the transitional zone and no immunoreactive fibres in the aganglionic segment. Comparative studies with antineuropeptide antibodies and with anti-neuronal cell antibodies suggest that D7 identifies a 52 kD antigen associated with a subset of axonal fibres distributed throughout the entire nervous system. These findings indicate that fundamental pathology in Hirschsprung's disease is not only the absence of ganglion cells of the myenteric and submucous plexuses but also the absence of D7 immunoreactive fibres in the circular muscle of the colon.
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Fujimoto, T., Reen, D. & Puri, P. 47 ABSENCE OF A NOVEL NEURONAL CELL ANTIGEN IN HIRSCHSPRUNG'S DISEASE. Pediatr Res 20, 1041 (1986). https://doi.org/10.1203/00006450-198610000-00101
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DOI: https://doi.org/10.1203/00006450-198610000-00101