Document Detail

Abnormalities in the distribution of laminin and collagen type IV in Hirschsprung's disease.
MedLine Citation:
PMID:  1551530     Owner:  NLM     Status:  MEDLINE    
In vitro neurite outgrowth and neuronal survival are promoted by laminin, and neuronal migration is promoted by collagen type IV. This led to the hypothesis that Hirschsprung's disease (HD) could be the result of an abnormal extracellular matrix microenvironment in the affected bowel during embryogenesis. Using indirect immunohistochemistry, we studied the distribution of laminin and collagen type IV in the bowel specimens of eight HD patients (four neonates and four infants) and 16 age- and site-matched controls from non-HD patients. In eight HD specimens, the tissue studied was from the aganglionic, transitional, and proximal normoganglionic zones. Uniform distribution of laminin and collagen type IV was observed in the basement membranes of all control specimens. The semiquantitative abnormality in the distribution of these proteins in HD occurred as follows: immunoreactivities were more intense in the inner circular layer, diminishing with a gradient in the outer muscularis externa in six of six aganglionic, four of five transitional, and three of eight normoganglionic sections. The qualitative abnormality of these proteins in HD was speckled immunofluorescence outside the basement membranes of the muscularis externa in all three zones of neonatal specimens only. These findings support the microenvironment hypothesis of the pathogenesis of HD.
D H Parikh; P K Tam; D Van Velzen; D Edgar
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Gastroenterology     Volume:  102     ISSN:  0016-5085     ISO Abbreviation:  Gastroenterology     Publication Date:  1992 Apr 
Date Detail:
Created Date:  1992-04-27     Completed Date:  1992-04-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0374630     Medline TA:  Gastroenterology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1236-41     Citation Subset:  AIM; IM    
Department of Child Health, Royal Liverpool Children's Hospital, England.
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MeSH Terms
Basement Membrane / chemistry
Collagen / analysis*
Extracellular Matrix / chemistry
Hirschsprung Disease / metabolism*
Infant, Newborn
Intestines / chemistry*
Laminin / analysis*
Reg. No./Substance:
0/Laminin; 9007-34-5/Collagen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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