TY - JOUR
T1 - Hirschsprung disease is linked to defects in neural crest stem cell function
AU - Iwashita, Toshihide
AU - Kruger, Genevieve M.
AU - Pardal, Ricardo
AU - Kiel, Mark J.
AU - Morrison, Sean J.
PY - 2003/8/15
Y1 - 2003/8/15
N2 - Genes associated with Hirschsprung disease, a failure to form enteric ganglia in the hindgut, were highly up-regulated in gut neural crest stem cells relative to whole-fetus RNA. One of these genes, the glial cell line-derived neurotrophic factor (GDNF) receptor Ret, was necessary for neural crest stem cell migration in the gut. GDNF promoted the migration of neural crest stem cells in culture but did not affect their survival or proliferation. Gene expression profiling, combined with reverse genetics and analyses of stem cell function, suggests that Hirschsprung disease is caused by defects in neural crest stem cell function.
AB - Genes associated with Hirschsprung disease, a failure to form enteric ganglia in the hindgut, were highly up-regulated in gut neural crest stem cells relative to whole-fetus RNA. One of these genes, the glial cell line-derived neurotrophic factor (GDNF) receptor Ret, was necessary for neural crest stem cell migration in the gut. GDNF promoted the migration of neural crest stem cells in culture but did not affect their survival or proliferation. Gene expression profiling, combined with reverse genetics and analyses of stem cell function, suggests that Hirschsprung disease is caused by defects in neural crest stem cell function.
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U2 - 10.1126/science.1085649
DO - 10.1126/science.1085649
M3 - Article
C2 - 12920301
AN - SCOPUS:0041520957
SN - 0036-8075
VL - 301
SP - 972
EP - 976
JO - Science
JF - Science
IS - 5635
ER -