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The expression pattern of laminin isoforms in Hirschsprung disease reveals a distal peripheral nerve differentiation

Alpy, F; Ritie, U; Jaubert, F; Becmeur, F; Mechine-Neuville, A; Lefebvre, O; Arnold, C; Sorokin, Lydia LU ; Kedinger, M and Simon-Assmann, P (2005) In Human Pathology 36(10). p.1055-1065
Abstract
Hirschsprung disease (HD), a developmental disorder, is associated with failure of enteric ganglia formation. Signaling molecules, including secreted basement membrane molecules, derived from the mesenchyme of the gut wall play an important role in the colonization and/or differentiation of the enteric nervous system. The current study aims to define the possible alterations of laminins involved in the pathogenesis of HD. Expression of the various laminin alpha, beta, and gamma chains, was assessed in the aganglionic, transitional, and ganglionic bowel segments of patients with HD or with other motor disorders. Cytoskeletal, neuronal, and glial markers were also included in this study. The major finding highlighted by the present work... (More)
Hirschsprung disease (HD), a developmental disorder, is associated with failure of enteric ganglia formation. Signaling molecules, including secreted basement membrane molecules, derived from the mesenchyme of the gut wall play an important role in the colonization and/or differentiation of the enteric nervous system. The current study aims to define the possible alterations of laminins involved in the pathogenesis of HD. Expression of the various laminin alpha, beta, and gamma chains, was assessed in the aganglionic, transitional, and ganglionic bowel segments of patients with HD or with other motor disorders. Cytoskeletal, neuronal, and glial markers were also included in this study. The major finding highlighted by the present work concerns the clear identification and location of myenteric aganglionic plexuses in HD with some of the laminin antibodies, which reveal a peripheral nerve type of differentiation. Furthermore, we could show an increase of laminin alpha 5 chain immunostaining in the dilated muscle of the ganglionic bowel upstream the distal aganglionic region in a subgroup of patients with HD, as well as a relocalization of laminin alpha 2 chain in the subepithelial basement membrane. Overall, these basement membrane molecules could provide useful markers for diagnosis of aganglionosis or hypoganglionosis. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Hirschsprung disease, laminins, development
in
Human Pathology
volume
36
issue
10
pages
1055 - 1065
publisher
Elsevier
external identifiers
  • pmid:16226104
  • wos:000232825600003
  • scopus:26644445705
ISSN
1532-8392
DOI
10.1016/j.humpath.2005.07.013
language
English
LU publication?
yes
id
625aba1c-bd14-4226-899e-74a3418706ed (old id 216571)
date added to LUP
2007-09-13 16:50:05
date last changed
2017-01-01 04:59:21
@article{625aba1c-bd14-4226-899e-74a3418706ed,
  abstract     = {Hirschsprung disease (HD), a developmental disorder, is associated with failure of enteric ganglia formation. Signaling molecules, including secreted basement membrane molecules, derived from the mesenchyme of the gut wall play an important role in the colonization and/or differentiation of the enteric nervous system. The current study aims to define the possible alterations of laminins involved in the pathogenesis of HD. Expression of the various laminin alpha, beta, and gamma chains, was assessed in the aganglionic, transitional, and ganglionic bowel segments of patients with HD or with other motor disorders. Cytoskeletal, neuronal, and glial markers were also included in this study. The major finding highlighted by the present work concerns the clear identification and location of myenteric aganglionic plexuses in HD with some of the laminin antibodies, which reveal a peripheral nerve type of differentiation. Furthermore, we could show an increase of laminin alpha 5 chain immunostaining in the dilated muscle of the ganglionic bowel upstream the distal aganglionic region in a subgroup of patients with HD, as well as a relocalization of laminin alpha 2 chain in the subepithelial basement membrane. Overall, these basement membrane molecules could provide useful markers for diagnosis of aganglionosis or hypoganglionosis.},
  author       = {Alpy, F and Ritie, U and Jaubert, F and Becmeur, F and Mechine-Neuville, A and Lefebvre, O and Arnold, C and Sorokin, Lydia and Kedinger, M and Simon-Assmann, P},
  issn         = {1532-8392},
  keyword      = {Hirschsprung disease,laminins,development},
  language     = {eng},
  number       = {10},
  pages        = {1055--1065},
  publisher    = {Elsevier},
  series       = {Human Pathology},
  title        = {The expression pattern of laminin isoforms in Hirschsprung disease reveals a distal peripheral nerve differentiation},
  url          = {http://dx.doi.org/10.1016/j.humpath.2005.07.013},
  volume       = {36},
  year         = {2005},
}