Transgenic rescue of aganglionosis and piebaldism in lethal spotted mice

Rice, Julie; Doggett, Barbara; Sweetser, David A.; Yanagisawa, Hiromi; Yanagisawa, Masashi; Kapur, Raj P.

doi:10.1002/(sici)1097-0177(200001)217:1<120::aid-dvdy11>3.0.co;2-u

Cited by 37 publications

(25 citation statements)

References 62 publications

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“…The authors propose that EDNRB plays a critical role in initiating emigration of melanoblasts and surviving the transitional period (78) (77), and that observed pigmentation differences may be explained by partial compensation of EDN3 deficiency (Edn3 ls /Edn3 ls ) by EDN1 and/or EDN2. Another report proposes that EDNRB-mediated signaling, essential immediately prior to and throughout the arrival of post-migratory neuroblasts in the gut (81). These studies are consistent with a proposed role for EDN3 signaling, in the maintenance of a pool of melanoblast and enteric neuroblast precursors.…”

Section: /Ednrbsupporting

confidence: 61%

EDNRB/EDN3 and Hirschsprung Disease Type II

McCallion

Chakravarti

2001

Pigment Cell Research

100

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Section: /Ednrbsupporting

confidence: 61%

EDNRB/EDN3 and Hirschsprung Disease Type II

McCallion

Chakravarti

2001

Pigment Cell Research

100

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“…Homozygous mutations of the genes that encode the receptor tyrosine kinase, Ret, or its ligand, glial cell-derived neurotrophic factor (gdnf), produce aganglionosis in mice (52,126). The mutation of ednrB or edn3, which encodes a ligand to ednrB, also causes distal intestinal aganglionosis in rodents and humans (75).…”

Section: Pertinent Background On Topics Essential To Evaluate the Rolmentioning

confidence: 99%

Are interstitial cells of Cajal plurifunction cells in the gut?

Sarna¹

2008

American Journal of Physiology-Gastrointestinal and Liver Physi

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The proposed functions of the interstitial cells of Cajal (ICC) are to 1) pace the slow waves and regulate their propagation, 2) mediate enteric neuronal signals to smooth muscle cells, and 3) act as mechanosensors. In addition, impairments of ICC have been implicated in diverse motility disorders. This review critically examines the available evidence for these roles and offers alternate explanations. This review suggests the following: 1) The ICC may not pace the slow waves or help in their propagation. Instead, they may help in maintaining the gradient of resting membrane potential (RMP) through the thickness of the circular muscle layer, which stabilizes the slow waves and enhances their propagation. The impairment of ICC destabilizes the slow waves, resulting in attenuation of their amplitude and impaired propagation.2) The one-way communication between the enteric neuronal varicosities and the smooth muscle cells occurs by volume transmission, rather than by wired transmission via the ICC. 3) There are fundamental limitations for the ICC to act as mechanosensors.4) The ICC impair in numerous motility disorders. However, a cause-and-effect relationship between ICC impairment and motility dysfunction is not established. The ICC impair readily and transform to other cell types in response to alterations in their microenvironment, which have limited effects on motility function. Concurrent investigations of the alterations in slow-wave characteristics, excitationcontraction and excitation-inhibition couplings in smooth muscle cells, neurotransmitter synthesis and release in enteric neurons, and the impairment of the ICC are required to understand the etiologies of clinical motility disorders. smooth muscle; slow waves; enteric neurons; excitation-contraction coupling; peristaltic reflex; motility disorders; volume transmission; synaptic transmission, ICC THE INTERSTITIAL CELLS WERE characterized originally by Raymond V. Cajal (20,21). These cells got his attention and that of others because they are intercalated between the autonomic nerves and the effector cells. On the basis of the understanding of the interneuronal communication in the central nervous system (CNS) at that time, Cajal proposed that the intercalating cells [later called the interstitial cells of Cajal (ICC)] mediate the transmission of signals from the autonomic neurons to the smooth muscle cells. A few years later, Keith (84) observed a collar of similar cells in the myenteric plexus of the rat ileocecal junction. He was looking for a pacemaker of the gut smooth muscle electrical activity because a few years earlier he had identified such a pacemaker in the sinoatrial node of the heart. He hypothesized that those interstitial cells were the pacemaker cells in the gut. In the late 1960s and early 1970s, several investigators described a plexus of the ICC within the myenteric and submucosal plexi of the small and large intestines. They also identified close connections between the enteric axonal varicosities and ICC processes on one side and g...

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“…Mice carrying the Edn3 ls allele (Lyon, 1996) were obtained from the Medical Research Council Mammalian Genetics Unit (Harwell, UK) and backcrossed to C57Bl/6 mice for at least six generations. Heterozygous and homozygous Edn3 ls animals were identified by PCR (Rice et al, 2000). The generation of Tg Wnt1Cre and R26 YFPStop animals have been described previously (Danielian et al, 1998;Srinivas et al, 2001).…”

Section: Animalsmentioning

confidence: 99%

Maintenance of mammalian enteric nervous system progenitors by SOX10 and endothelin 3 signalling

Bondurand¹,

Natarajan²,

Barlow³

et al. 2006

Development

152

128

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The transcriptional regulator SOX10 and the signalling molecule endothelin 3 have important roles in the development of the mammalian enteric nervous system (ENS). Using a clonal cell culture system, we show that SOX10 inhibits overt neuronal and glial differentiation of multilineage ENS progenitor cells (EPCs), without interfering with their neurogenic commitment. We also demonstrate that endothelin 3 inhibits reversibly the commitment and differentiation of EPCs along the neurogenic and gliogenic lineages, suggesting a role for this factor in the maintenance of multilineage ENS progenitors. Consistent with such a role, the proportion of Sox10-expressing progenitors in the total population of enteric neural crest cells is reduced in the gut of endothelin 3-deficient embryos. This reduction may be related to the requirement of endothelin signalling for the proliferation of ENS progenitors. The dependence of ENS progenitors on endothelin 3 is more pronounced at the migratory front of enteric neural crest cells, which is associated with relatively high levels of endothelin 3 mRNA. Our findings indicate that SOX10 and endothelin 3 have a crucial role in the maintenance of multilineage enteric nervous system progenitors.

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Transgenic rescue of aganglionosis and piebaldism in lethal spotted mice

Cited by 37 publications

References 62 publications

EDNRB/EDN3 and Hirschsprung Disease Type II

EDNRB/EDN3 and Hirschsprung Disease Type II

Are interstitial cells of Cajal plurifunction cells in the gut?

Maintenance of mammalian enteric nervous system progenitors by SOX10 and endothelin 3 signalling

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