Brain size reductions associated with endothelin B receptor mutation, a cause of Hirschsprung's disease.

ABSTRACT:

Background

ET_B has been reported to regulate neurogenesis and vasoregulation in foetal development. Its dysfunction was known to cause HSCR, an aganglionic colonic disorder with syndromic forms reported to associate with both small heads and developmental delay. We therefore asked, "is CNS maldevelopment a more general feature of ET_B mutation?" To investigate, we reviewed the micro-CT scans of an ET_B^-/- model animal, sl/sl rat, and quantitatively evaluated the structural changes of its brain constituents.

Methods

Eleven neonatal rats generated from ET_B^+/- cross breeding were sacrificed. Micro-CT scans were completed following 1.5% iodine-staining protocols. All scans were reviewed for morphological changes. Se

SUBMITTER: Chen KC 

PROVIDER: S-EPMC8214790 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature