O’Donnell AM, Coyle D, Puri P. Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung’s disease. World J Gastroenterol 2015; 21(18): 5635-5640 [PMID: 25987789 DOI: 10.3748/wjg.v21.i18.5635]
Corresponding Author of This Article
Prem Puri, Professor, MS, FRCS, FRCS (Ed), FACS, FAAP (Hon.), DSc (Hon.), National Children’s Research Centre, Our Lady’s Children’s Hospital, Cooley Rd, Crumlin, Dublin 12, Ireland. prem.puri@ucd.ie
Research Domain of This Article
Surgery
Article-Type of This Article
Clinical Trials Study
Open-Access Policy of This Article
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World J Gastroenterol. May 14, 2015; 21(18): 5635-5640 Published online May 14, 2015. doi: 10.3748/wjg.v21.i18.5635
Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung’s disease
Anne Marie O’Donnell, David Coyle, Prem Puri
Anne Marie O’Donnell, David Coyle, Prem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Ireland
Author contributions: O’Donnell AM and Puri P contributed to study conception; O’Donnell AM and Coyle D performed the experiments; all authors contributed to drafting the manuscript; O’Donnell AM and Puri P drafted the final manuscript.
Supported by National Children’s Research Centre/Children’s Medical Research Foundation, Ireland.
Ethics approval: The study was reviewed and approved by the Temple Street Children’s University Hospital Research Ethics Committee No. 13.003.
Clinical trial registration: No clinical trial registration information is available for this study.
Informed consent: Informed written consent for participation in the study was provided by all parents and legal guardians of participants prior to enrollment in the study.
Conflict-of-interest: The authors declare that they have no conflict of interest.
Data sharing: Dataset is available from the corresponding author at prem.puri@ncrc.ie.
Correspondence to: Prem Puri, Professor, MS, FRCS, FRCS (Ed), FACS, FAAP (Hon.), DSc (Hon.), National Children’s Research Centre, Our Lady’s Children’s Hospital, Cooley Rd, Crumlin, Dublin 12, Ireland. prem.puri@ucd.ie
Telephone: +353-1-4096420 Fax: +353-1-4550201
Received: October 9, 2014 Peer-review started: October 9, 2014 First decision: November 14, 2014 Revised: November 28, 2014 Accepted: December 19, 2014 Article in press: December 22, 2014 Published online: May 14, 2015 Processing time: 221 Days and 16.8 Hours
Core Tip
Core tip: Children with Hirschsprung’s disease experience symptoms of intestinal obstruction due to a spastically contracted aganglionic segment of distal bowel, however the mechanism for this spasticity is incompletely understood. Hyperpolarisation-activated nucleotide-gated channels play a key role in regulating cell excitability in both pacemaker and non-pacing cells. We examined expression of hyperpolarisation-activated nucleotide-gated (HCN) 1-4 in both Hirschsprung’s disease (HSCR) colon and healthy controls. While HCN-1 expression was absent, and HCN-2 and HCN-4 were expressed at similar levels in diseased and healthy bowel, we found HCN-3 to be reduced in aganglionic bowel in HSCR. We suggest deficient HCN3 channels may be involved in the pathophysiology of HSCR.
