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Article

Hedgehog/Notch-induced premature gliogenesis represents a new disease mechanism for Hirschsprung disease in mice and humans.

The Journal of clinical investigation - 1 Sept 2011

Ngan Elly Sau-Wai, Garcia-Barceló Maria-Mercè, Yip Benjamin Hon-Kei, Poon Hiu-Ching, Lau Sin-Ting, Kwok Carmen Ka-Man, Sat Eric, Sham Mai-Har, Wong Kenneth Kak-Yuen, Wainwright Brandon J, Cherny Stacey S, Hui Chi-Chung, Sham Pak Chung, Lui Vincent Chi-Hang, Tam Paul Kwong-Hang

Abstract excerpt

Hirschsprung (HSCR) disease is a complex genetic disorder attributed to a failure of the enteric neural crest cells (ENCCs) to form ganglia in the hindgut. Hedgehog and Notch are implicated in mediating proliferation and differentiation of ENCCs. Nevertheless, how these signaling molecules may interact to mediate gut colonization by ENCCs and contribute to a primary etiology for HSCR are not known. Here, we...

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Source

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Identifiers

PMID: 21841314
DOI: 10.1172/JCI43737
PMCID: PMC3163945

Genes

DLL3
PTCH1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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