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Hedgehog in the human: A possible explanation for the VATER association
Author(s) -
Arsic D,
Qi BQ,
Beasley SW
Publication year - 2002
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1046/j.1440-1754.2002.00813.x
Subject(s) - foregut , sonic hedgehog , medicine , morphogenesis , hedgehog , hedgehog signaling pathway , gli3 , association (psychology) , anatomy , pathology , microbiology and biotechnology , signal transduction , biology , genetics , gene , philosophy , epistemology , repressor , transcription factor
: Foregut malformations are relatively common anomalies, occurring in 1 in 2000–5000 live births. The adriamycin‐induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with VATER association. The secreted glycoprotein, Sonic hedgehog (Shh), may act as an endodermal signal that controls gut and lung patterning. Mice with targeted deletion of Shh have foregut defects that are consistent with those produced by administration of adriamycin. It is possible that mutations induced by adriamycin may result from the breakdown of the Shh signalling pathway.