PMID- 31939867 OWN - NLM STAT- MEDLINE DCOM- 20210618 LR - 20210618 IS - 1536-4801 (Electronic) IS - 0277-2116 (Linking) VI - 70 IP - 4 DP - 2020 Apr TI - Aberrant Distributions of Collagen I, III, and IV in Hirschsprung Disease. PG - 450-456 LID - 10.1097/MPG.0000000000002627 [doi] AB - BACKGROUND: Hirschsprung disease (HSCR) is the most common congenital gut motility disorder, involving a severe anomaly of the enteric nervous system, and is characterized by functional intestinal obstruction due to lack of intrinsic innervation (aganglionosis) in the distal bowel. OBJECTIVE: The aim of this study was to examine the distribution patterns of collagens I (Col I), III (Col III), and IV (Col IV) in the enteric nervous system of HSCR patients, to determine whether or not collagen levels are altered in the aganglionic bowel. METHODS: We measured the expression levels of Col I, Col III, and Col IV in colonic muscle from 129 children with HSCR. The localizations of the 3 collagens and myenteric ganglia were assessed morphologically by immunofluorescence staining. western blots and real-time fluorescence quantitative polymerase chain reaction were performed to examine the relative levels of these collagens in aganglionic, transitional, and ganglionic colon segments. RESULTS: Immunoreactivities of Col I and Col III were high around and within myenteric ganglia in the ganglionic segment, moderate in the transitional segment, and weak in the aganglionic segment. Col IV immunoreactivity showed the opposite pattern, being lowest in the ganglionic segment and highest in the aganglionic segment. CONCLUSION: Col I and Col III are decreased and Col IV is increased in the distal colon of patients with HSCR. FAU - Gao, Ni AU - Gao N AD - Department of Pediatric Surgery, Qilu Hospital, Shandong University, Jinan, Shandong Province, China. FAU - Wang, Jian AU - Wang J FAU - Zhang, Qiangye AU - Zhang Q FAU - Zhou, Tingting AU - Zhou T FAU - Mu, Weijing AU - Mu W FAU - Hou, Peimin AU - Hou P FAU - Wang, Dongming AU - Wang D FAU - Lv, Xiaona AU - Lv X FAU - Li, Aiwu AU - Li A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Pediatr Gastroenterol Nutr JT - Journal of pediatric gastroenterology and nutrition JID - 8211545 SB - IM MH - Blotting, Western MH - Child MH - Colon MH - *Hirschsprung Disease/genetics MH - Humans MH - Infant MH - Intestines MH - Real-Time Polymerase Chain Reaction EDAT- 2020/01/16 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/01/16 06:00 PHST- 2020/01/16 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/01/16 06:00 [entrez] AID - 00005176-202004000-00015 [pii] AID - 10.1097/MPG.0000000000002627 [doi] PST - ppublish SO - J Pediatr Gastroenterol Nutr. 2020 Apr;70(4):450-456. doi: 10.1097/MPG.0000000000002627.