Surgical Intervention to Rescue Hirschsprung Disease in a Rat Model
Lincon A Stamp, Florian Obermayr, Louise Pontell, Heather M Young, Dan Xie, David H Croaker, Zan-Min Song, John B Furness
JOURNAL OF NEUROGASTROENTEROLOGY AND MOTILITY | KOREAN SOC NEUROGASTROENTEROLOGY & MOTILITY | Published : 2015
BACKGROUND/AIMS: Rats with a spontaneous null mutation in endothelin receptor type B or Ednrb (sl/sl; spotting lethal) lack enteric neurons in the distal bowel and usually die within the first week after birth. This early postnatal lethality limits their use for examining the potential of cell therapy to treat Hirschsprung disease, and for studies of the influence of EDNRB on the mature CNS and vascular systems. METHODS: We have developed a surgical intervention to prolong the life of the spotting lethal sl/sl rat, in which we perform a colostomy on postnatal (P) day 4-6 rats to avoid the fatal obstruction caused by the lack of colonic enteric neurons. RESULTS: The stomas remained patent and..View full abstract
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Awarded by NHMRC
This work was funded by NHMRC Project grants (No.1019931 and 1079234) to Heather M Young, Lincon A Stamp, and John B Furness. We also acknowledge the assistance of a donation from The Bootes Foundation to Zan-Min Song and David H Croaker.