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Updated: May 9, 2026

Visualization of the Interstitial Cells of Cajal (ICC) Network in Mice
Published on: July 27, 2011
Interstitial cells of Cajal in the normal human gut and in Hirschsprung disease
1Department of Paediatric Surgery, University Hospital, Goethe University Frankfurt/M, 60596 Frankfurt/M, Germany, Stefan.Gfroerer@kgu.de
Insights
Hirschsprung disease (HD) involves impaired gut innervation. This review explores how interstitial cells of Cajal (ICC) dysfunction contributes to motility issues in HD patients.
Area of Science:
- Gastroenterology and Developmental Biology
Background:
- Hirschsprung disease (HD) is the most common congenital gastrointestinal motility disorder.
- It results from defective intrinsic innervation of the distal bowel, causing functional intestinal obstruction.
- Interstitial cells of Cajal (ICC) are crucial for gastrointestinal peristalsis and motility.
Purpose of the Study:
- To review the distribution and functions of ICCs in the normal gut.
- To examine the role of ICCs in the pathogenesis of Hirschsprung disease.
Main Methods:
- Literature review focusing on the role of ICCs in gastrointestinal motility.
- Analysis of studies detailing ICC distribution and function in both healthy and HD conditions.
Main Results:
- ICCs are specialized pacemaker cells generating slow waves essential for peristalsis.
- ICCs regulate mechanical activity and neurotransmission in the gut.
- Impairments in ICCs are implicated in motility dysfunction, including in Hirschsprung disease.
Conclusions:
- Understanding ICCs' role is vital for elucidating Hirschsprung disease pathogenesis.
- Defects in ICCs likely contribute significantly to the motility dysfunction observed in HD.
- Further research into ICCs may reveal new therapeutic targets for HD.
Abstract:
Hirschsprung disease (HD) is the most prevalent congenital gastrointestinal motility disorder. The pathogenesis of HD is defined as a functional intestinal obstruction resulting from a defect in the intrinsic innervation of the distal bowel. In addition to the enteric nervous system, the interstitial cells of Cajal (ICC) play an important role in the generation of coordinated gastrointestinal peristalsis. The major function of the ICCs is the generation of slow waves that allow these cells to act as specialised pacemaker cells within various tissues. ICCs have additional functions in the gastrointestinal tract as regulators of mechanical activity and neurotransmission. Due to the central role of ICCs in gastrointestinal peristalsis, it has been suggested that defects or impairments of the ICCs may contribute to motility dysfunction in several gastrointestinal motility disorders. This review describes the distribution and functions of ICCs in the normal gut and in Hirschsprung disease.
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