37/67-laminin receptor facilitates neural crest cell migration during enteric nervous system development.

ABSTRACT: Enteric nervous system (ENS) development is governed by interactions between neural crest cells (NCC) and the extracellular matrix (ECM). Hirschsprung disease (HSCR) results from incomplete NCC migration and failure to form an appropriate ENS. Prior studies implicate abnormal ECM in NCC migration failure. We performed a comparative microarray of the embryonic distal hindgut of wild-type and EdnrB^NCC-/- mice that model HSCR and identified laminin-β1 as upregulated in EdnrB^NCC-/- colon. We identified decreased expression of 37/67 kDa laminin receptor (LAMR), which binds laminin-β1, in human HSCR myenteric plexus and EdnrB^NCC-/- NCC. Using a combination of in vitro gut slice cultures and ex vivo organ cultures, we determined the mechanistic role of LAMR in NCC

SUBMITTER: Fu M 

PROVIDER: S-EPMC7759089 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature