Altered neuronal density and neurotransmitter expression in the ganglionated region of Ednrb null mice: implications for Hirschsprung's disease.

ABSTRACT:

Background

Hirschsprung's disease (HSCR) is a congenital condition in which enteric ganglia, formed from neural crest cells (NCC), are absent from the terminal bowel. Dysmotility and constipation are common features of HSCR that persist following surgical intervention. This persistence suggests that the portion of the colon that remains postoperatively is not able to support normal bowel function. To elucidate the defects that underlie this condition, we utilized a murine model of HSCR.

Methods

Mice with NCC-specific deletion of Ednrb were used to measure the neuronal density and neurotransmitter expression in ganglia.

Key results

At the site located proximal to the aganglionic region of P21 Ednrb null mice, the neuronal density is significantly decreased and the expr

SUBMITTER: Zaitoun I 

PROVIDER: S-EPMC3578114 | biostudies-literature | 2013 Mar

REPOSITORIES: biostudies-literature