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Article

Transgenic expression of the endothelin-B receptor prevents congenital intestinal aganglionosis in a rat model of Hirschsprung disease.

15 Sept 1998

Abstract excerpt

The spotting lethal rat, a naturally occurring rodent model of Hirschsprung disease, carries a deletion in the endothelin-B receptor (EDNRB) gene that abrogates expression of functional EDNRB receptors. Rats homozygous for this mutation (sl) exhibit coat color spotting and congenital intestinal aganglionosis. These deficits result from failure of the neural crest-derived epidermal melanoblasts and enteric nervous...

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Identifiers

PMID: 9739043
DOI: 10.1172/jci3702

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Transgenic expression of the endothelin-B receptor prevents congenital intestinal aganglionosis in a rat model of Hirschsprung disease.PMID 9739043Read ↗

Null mutation of endothelin receptor type B gene in spotting lethal rats causes aganglionic megacolon and white coat color.Semantic proximity Read ↗

A mutation in endothelin-B receptor gene causes myenteric aganglionosis and coat color spotting in rats.Semantic proximity Read ↗

Targeted and natural (piebald-lethal) mutations of endothelin-B receptor gene produce megacolon associated with spotted coat color in mice.Semantic proximity Read ↗

Targeting of endothelin receptor‐B to the neural crestSemantic proximity Read ↗

Intercellular signals downstream of endothelin receptor-B mediate colonization of the large intestine by enteric neuroblasts.Semantic proximity Read ↗

Abnormalities of the enteric nervous system in heterozygous endothelin B receptor deficient (spotting lethal) rats resembling intestinal neuronal dysplasia.Semantic proximity Read ↗

Colostomy delays cell loss in the brain and improves juvenile survival in a neonatal rat model of Hirschsprung’s diseaseSemantic proximity Read ↗

Interaction of endothelin-3 with endothelin-B receptor is essential for development of epidermal melanocytes and enteric neurons.Semantic proximity Read ↗

Hirschsprung's disease: genetic mutations in mice and men.Semantic proximity Read ↗

Interstitial deletion of the endothelin-B receptor gene in the spotting lethal (sl) rat.Semantic proximity Read ↗

Cell-autonomous and cell non-autonomous signaling through endothelin receptor B during melanocyte development.Semantic proximity Read ↗

Molecular characterization of four induced alleles at the Ednrb locus.Semantic proximity Read ↗

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