The Role of Interstitial Cells of Cajal (ICC) in Gastrointestinal Motility Disorders - What the Gastroenterologist Has to Know

Breuer, Christian

doi:10.5772/28297

Cited by 2 publications

(4 citation statements)

References 48 publications

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“…Beyond CIPO, altered numbers / depletion, distribution, morphology or ultrastructural changes were reported to occur in ICC/ ICC networks located in various parts of the gut in several chronic gastrointestinal motility disorders, including the esophagus (achalasia and gastroesophageal reflux), stomach (diabetic gastroenteropathy and infantile hypertrophic pyloric stenosis) and in patients with genetic alterations in kit ; in the small intestine and colon (Hirschsprung’s disease, idiopathic megacolon, juvenile intestinal pseudo-obstruction, anorectal malformations, slow transit constipation) and in inflammatory bowel diseases (ulcerative colitis and Crohn’s disease) [35, 55, 56].…”

Section: Interstitial Cells Of Cajal (Icc)mentioning

confidence: 99%

“…Evaluation of non- invasive rectal tissue by routine histology would provide a valuable diagnostic tool which shows megamitochondria readily observed as cytoplasmic eosinophilic inclusions by light microscopy [25]. On the other hand, the strong correlation between Kit immunoreactivity and ICC facilitated diagnosis of human GI motility disorders, and due to their role, ICC are suggested to be included as a diagnostic criterion for patients with symptoms of pseudo-obstruction [55]. Immunohistochemistry detection of c-kit+ ICC has been applied for this purpose.…”

Section: Future Prospectsmentioning

confidence: 99%

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Gastrointestinal Dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal

Yadak

Breur

Bugiani

2019

Orphanet J Rare Dis

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BackgroundMNGIE is a rare and fatal disease in which absence of the enzyme thymidine phosphorylase induces systemic accumulation of thymidine and deoxyuridine and secondary mitochondrial DNA alterations. Gastrointestinal (GI) symptoms are frequently reported in MNGIE patients, however, they are not resolved with the current treatment interventions.Recently, our understanding of the GI pathology has increased, which rationalizes the pursuit of more targeted therapeutic strategies. In particular, interstitial cells of Cajal (ICC) play key roles in GI physiology and are involved in the pathogenesis of the GI dysmotility. However, understanding of the triggers of ICC deficits in MNGIE is lacking. Herein, we review the current knowledge about the pathology of GI dysmotility in MNGIE, discuss potential mechanisms in relation to ICC loss/dysfunction, remark on the limited contribution of the current treatments, and propose intervention strategies to overcome ICC deficits. Finally, we address the advances and new research avenues offered by organoids and tissue engineering technologies, and propose schemes to implement to further our understanding of the GI pathology and utility in regenerative and personalized medicine in MNGIE.ConclusionInterstitial cells of Cajal play key roles in the physiology of the gastrointestinal motility. Evaluation of their status in the GI dysmotility related to MNGIE would be valuable for diagnosis of MNGIE. Understanding the underlying pathological and molecular mechanisms affecting ICC is an asset for the development of targeted prevention and treatment strategies for the GI dysmotility related to MNGIE.

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Section: Interstitial Cells Of Cajal (Icc)mentioning

confidence: 99%

Section: Future Prospectsmentioning

confidence: 99%

Gastrointestinal Dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal

Yadak

Breur

Bugiani

2019

Orphanet J Rare Dis

View full text Add to dashboard Cite

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“…Lower total ICC count in age-matched NZ compared with control in the present study was related to lower ICC count in the muscular coat, while differences in the ICC-SP and ICC-MP were not statistically significant. The depletion of the ICC in the muscular layers of the gut may contribute to the inability of relaxation of the smooth muscle (6) and may contribute to postoperative constipation.…”

Section: Discussionmentioning

confidence: 99%

“…Motility of gastrointestinal tract is controlled by the enteric nervous system and interstitial cells of Cajal (ICC). ICC produce electrical impulses which cause slow peristaltic contraction (6). They have mesodermal origin and express tyrosine-kinase receptor, which is recognised by c-Kit antibody (CD117).…”

Section: Introductionmentioning

confidence: 99%

Altered Distribution of Interstitial Cells of Cajal in Normoganglionic and Transitional Zone of Hirschsprung Disease and Their Clinical Significance

Janković

Sindjic-Antunovic

Lukač

et al. 2020

CEJoP

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Objective − The aim of this study was to evaluate interstitial cells of Cajal (ICC) in Hirschsprung disease (HD), especially in children who had postsurgical problems. Material and Methods − The study included tissue samples of normoganglionic (NZ) and transitional zone (TZ) of 33 HD pediatric patients. Samples of bowel from 10 autopsy controls who did not have dysmotility were also analyzed. Hematoxylin-eosin (H&E) and immunohistochemical (MAP-2 and c-Kit (CD117)) staining were performed. Myenteric ganglia were analyzed at 5 microscopic fields H&E and MAP-2 stained sections (magnification 400×). The diameters of the submucosal nerves were measured at 3 microscopic H&E fields (magnification 400×). The ICC were analyzed on c-Kit immunostained slides at 3 microscopic fields per each bowel layer (magnification 200×): deep submucosa, circular and longitudinal muscular layer and zone of myenteric plexus. Results − The myenteric ganglion cell count was significantly lower in TZ of all children with HD, while there were no significant differences in NZ between the study group and age-matched controls. The ICC network was affected in all cases of HD. The total ICC count was significantly lower in NZ than in control (P<0.001). A critically lower ICC count (<50% of minimal ICC count in the control group) in the NZ was found in patients with postoperative problems (constipation and enterocolitis). Conclusion − Decreased count of the ICC in the NZ could be the cause of intestinal motility difficulties in the postoperative period in children with HD.

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The Role of Interstitial Cells of Cajal (ICC) in Gastrointestinal Motility Disorders - What the Gastroenterologist Has to Know

Cited by 2 publications

References 48 publications

Gastrointestinal Dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal

Gastrointestinal Dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal

Altered Distribution of Interstitial Cells of Cajal in Normoganglionic and Transitional Zone of Hirschsprung Disease and Their Clinical Significance

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