The interstitial cells of Cajal are mesenchymal cells closely apposed to neural and smooth muscle cells of the gut. They form a heterogeneous group with differing ultrastructure and functions. One cell type has an ancillary neural function as a gastrointestinal pacemaker, generating electrical slow waves that drive rhythmic smooth muscle contraction, both peristalsis for propulsion and segmentation for mixing. Failure of this network causes gastroparesis and intestinal pseudo-obstruction.
Related pathology
Gastrointestinal stromal tumors (GISTs) are a heterogeneous family of submucosal tumors derived from interstitial cells of Cajal or common progenitor cells. The oncogenic mutations (80% c-KIT and 20% PDGFRA genes) produce abnormal receptor tyrosine kinase proteins, which are always activated leading to uncontrolled cell proliferation. Extra-gastrointestinal GISTs arise from interstitial cells of Cajal in other organs.
History and Etymology
These cells are named after S. Ramon y Cajal, an eminent Spanish neuroanatomist in the 19th century who described the fibroblast-type interstitial cells of Cajal.
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