The transition zone in Hirschsprung's bowel contains abnormal hybrid ganglia with characteristics of extrinsic nerves



Smith, Megan ORCID: 0000-0002-0827-3295, Chhabra, Sumita, Shukla, Rajeev, Kenny, Simon, Almond, Sarah, Edgar, David and Wilm, Bettina ORCID: 0000-0002-9245-993X
(2023) The transition zone in Hirschsprung's bowel contains abnormal hybrid ganglia with characteristics of extrinsic nerves. JOURNAL OF CELLULAR AND MOLECULAR MEDICINE, 27 (2). pp. 287-298.

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Abstract

The aganglionic bowel in short-segment Hirschsprung's disease is characterized both by the absence of enteric ganglia and the presence of extrinsic thickened nerve bundles (TNBs). The relationship between the TNBs and the loss of enteric ganglia is unknown. Previous studies have described decreasing numbers of ganglia with increasing density of TNBs within the transition zone (TZ) between ganglionic and aganglionic gut, and there is some evidence of spatial contact between them in this region. To determine the cellular interactions involved, we have analysed the expression of perineurial markers of TNBs and enteric ganglionic markers for both neural cells and their ensheathing telocytes across four cranio-caudal segments consisting of most proximal ganglionic to most distal aganglionic from pull-through resected colon. We show that in the TZ, enteric ganglia are abnormal, being surrounded by perineurium cells characteristic of TNBs. Furthermore, short processes of ganglionic neurons extend caudally towards the aganglionic region, where telocytes in the TNB are located between the perineurium and nerve fibres into which they project telopodes. Thus, enteric ganglia within the TZ have abnormal structural characteristics, the cellular relationships of which are shared by the TNBs. These findings will help towards elucidation of the cellular mechanisms involved in the aetiology of Hirschsprung's disease.

Item Type: Article
Uncontrolled Keywords: CD34, enteric nervous system, extrinsic nerves, ganglia, Hirschsprung's disease, p75, telocytes, transition zone
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Systems, Molecular and Integrative Biology
Depositing User: Symplectic Admin
Date Deposited: 07 Dec 2022 11:26
Last Modified: 24 Jan 2023 11:05
DOI: 10.1111/jcmm.17659
Open Access URL: https://doi.org/10.1111/jcmm.17659
Related URLs:
URI: https://livrepository.liverpool.ac.uk/id/eprint/3166504