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Physiology and Pathophysiology of the Interstitial Cell of Cajal: From Bench to Bedside
Functional development and plasticity ofinterstitial cells of Cajal networks
Physiology
and Pathophysiology of the Interstitial Cell of Cajal: From
Bench to Bedside. I. Functional development and plasticity of
interstitial cells of Cajal networks. Am J Physiol Gastrointest
Liver Physiol 281: G602–G611, 2001.—Interstitial cells of
Cajal (ICC) are the pacemaker cells in gastrointestinal (GI)
muscles. They also mediate or transduce inputs from enteric
motor nerves to the smooth muscle syncytium. What is
known about functional roles of ICC comes from developmental
studies based on the discovery that ICC express c-kit.
Functional development of ICC networks depends on signaling
via the Kit receptor pathway. Immunohistochemical
studies using Kit antibodies have expanded our knowledge
about the ICC phenotype, the structure of ICC networks, the
interactions of ICC with other cells within the tunica muscularis,
and the loss of ICC in some motility disorders. Manipulating
Kit signaling with reagents to block the receptor
or downstream signaling pathways or by using mutant mice
in which Kit or its ligand, stem cell factor, are defective has
allowed novel studies of the development of these cells within
the tunica muscularis and also allowed the study of specific
functions of different classes of ICC in several regions of the
GI tract. This article examines the role of ICC in GI motility,
focusing on the functional development and maintenance of
ICC networks in the GI tract and the phenotypic changes
that can occur when the Kit signaling pathway is disrupted.
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