Human embryoid bodies as a 3D tissue model of the extracellular matrix and α-dystroglycanopathies.

ABSTRACT: The basal lamina is a specialized sheet of dense extracellular matrix (ECM) linked to the plasma membrane of specific cell types in their tissue context, which serves as a structural scaffold for organ genesis and maintenance. Disruption of the basal lamina and its functions is central to many disease processes, including cancer metastasis, kidney disease, eye disease, muscular dystrophies and specific types of brain malformation. The latter three pathologies occur in the α-dystroglycanopathies, which are caused by dysfunction of the ECM receptor α-dystroglycan. However, opportunities to study the basal lamina in various human disease tissues are restricted owing to its limited accessibility. Here, we report the generation of embryoid bodies from human induced pluripotent stem cells that m

SUBMITTER: Nickolls AR 

PROVIDER: S-EPMC7328151 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature