Altered distribution of small-conductance calcium-activated potassium channel SK3 in Hirschsprung's disease

Journal of Pediatric Surgery
David CoyleP Puri

Abstract

SK3 channels are voltage-independent Ca(2+)-dependent K(+) channels that play a key role in regulating smooth muscle membrane potential during purinergic inhibitory neurotransmission in the colon. Dysmotility problems are common after a properly performed pull-through operation for Hirschsprung's disease (HSCR). We hypothesised that ganglionic bowel just proximal to the transition zone is abnormal and designed this study to investigate SK3 channel expression in HSCR. Entire resected bowel specimens were collected at the time of pull-through surgery for HSCR (n=6). Control colonic specimens were obtained at the time of colostomy closure in patients following anorectoplasty (n=6). SK3 protein expression was assessed qualitatively using immunofluorescence with confocal microscopy and quantitatively using western blot (WB) analysis. Positive SK3 immunofluorescence was seen in the mucosa and in all three smooth muscle layers and the myenteric plexus in control specimens. SK3 immunofluorescence co-localised with PDGFRα. A band was detected at ~70kDa on WB. SK3 protein expression was barely detectable in aganglionic tissue and markedly reduced in the ganglionic bowel of 3 patients with HSCR compared to controls. Decreased SK3 expressi...Continue Reading

References

Jun 6, 2006·American Journal of Physiology. Gastrointestinal and Liver Physiology·Diana GallegoMarcel Jiménez
Oct 27, 2007·Pediatric Surgery International·Anne-Marie O' Donnell, Prem Puri
Feb 29, 2008·Biology of Reproduction·Stephanie L PierceSarah K England
Sep 10, 2008·Journal of Pediatric Surgery·Maria MenezesPrem Puri
Dec 22, 2010·The Journal of Physiology·Masaaki KurahashiKenton M Sanders
Nov 8, 2013·Journal of Neurogastroenterology and Motility·Sang Don Koh, Poong-Lyul Rhee

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Citations

Feb 15, 2018·BMC Medical Genetics· GunadiAndi Dwihantoro
Dec 14, 2019·BMC Pediatrics· GunadiAkhmad Makhmudi
Mar 29, 2020·Frontiers in Pediatrics· GunadiJoris A Veltman
Mar 30, 2016·World Journal of Gastroenterology : WJG·Anne-Marie O'DonnellPrem Puri

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