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Anorectal neural crest derived cell behavior after the migration of vagal neural crest derived cells is surgically disrupted: implications for the etiology of Hirschsprung's disease.
MedLine Citation:
PMID:  23143079     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND/PURPOSE: In Hirschsprung's disease (HD), thick extrinsic nerve fibers can be associated with the aganglionic segment in the anorectum. We surgically disrupted the migration of vagal neural crest cell-derived cells (vagal NCC) in embryos from transgenic mice we created previously (SOX10-VENUS Tg) which have the SOX10 gene labeled with Venus (V), a green fluorescent protein, to observe sacral NCC activity in the anorectum. METHOD: Proximal colon harvested from SOX10-VENUS Tg embryos on day 10.5 (n = 10) was transected at the ascending colon. V-positive sacral NCC in the anorectum were observed during organ culture under fluorescence stereoscopic microscopy, and compared with non-transected control specimens (n = 10). RESULTS: In transected specimens, no V-positive sacral NCC were identified initially in the anorectum. By day 2, there were thick beaded sacral NCC in the anorectum in 6/10 (60 %) that migrated steadily to the transected end over 3-4 days. In controls, thinner and shorter V-positive sacral NCC began migrating cranially on day 2, and were met by distally migrating vagal NCC. CONCLUSION: Disruption of vagal NCC migration appears to induce sacral NCC activity in the anorectum, suggesting that thick extrinsic nerve fibers seen in HD may be a secondary phenomenon.
Authors:
Katsumi Miyahara; Yoshifumi Kato; Ryota Suzuki; Chihiro Akazawa; Nana Tanaka; Hiroyuki Koga; Takashi Doi; Geoffrey J Lane; Atsuyuki Yamataka
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-11
Journal Detail:
Title:  Pediatric surgery international     Volume:  -     ISSN:  1437-9813     ISO Abbreviation:  Pediatr. Surg. Int.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8609169     Medline TA:  Pediatr Surg Int     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
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