Hirschsprung disease is linked to defects in neural crest stem cell function.
basic_science · Level V
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- Record sourced from PubMed, PMID 12920301.
- Also identified by PMC identifier 2614078.
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Abstract
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.
Medical subject headings
- Digestive System
- Gene Expression Regulation, Developmental
- Hirschsprung Disease
- Multipotent Stem Cells
- Neural Crest
- Proto-Oncogene Proteins
- Receptor Protein-Tyrosine Kinases