Journal
NEURON
Volume 40, Issue 5, Pages 917-929Publisher
CELL PRESS
DOI: 10.1016/S0896-6273(03)00727-X
Keywords
-
Categories
Funding
- NCI NIH HHS [T32 CA09676, CA46592] Funding Source: Medline
- NIAMS NIH HHS [P30 AR48310, AR20557] Funding Source: Medline
- NIA NIH HHS [AG00114-18] Funding Source: Medline
- NIDDK NIH HHS [P60DK-20572] Funding Source: Medline
- NINDS NIH HHS [R01 NS040750-01] Funding Source: Medline
Ask authors/readers for more resources
Loss of Endothelin-3/Endothelin receptor B (EDNRB) signaling leads to aganglionosis of the distal gut (Hirschsprung's disease), but it is unclear whether it is required primarily for neural crest progenitor maintenance or migration. Ednrb-deficient gut neural crest stem cells (NCSCs) were reduced to 40% of wild-type levels by embryonic day 12.5 (E12.5), but no further depletion of NCSCs was subsequently observed. Undifferentiated NCSCs persisted in the proximal guts of Ednrb-deficient rats throughout fetal and postnatal development but exhibited migration defects after E12.5 that prevented distal gut colonization. EDNRB signaling may be required to modulate the response of neural crest progenitors to migratory cues, such as glial cell line-derived neurotrophic factor (GDNF). This migratory defect could be bypassed by transplanting wild-type NCSCs directly into the aganglionic region of the Ednrb(sl/sl) gut, where they engrafted and formed neurons as efficiently as in the wild-type gut.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available