Ontology highlight
ABSTRACT:
SUBMITTER: Korogi Y
PROVIDER: S-EPMC6409438 | biostudies-literature | 2019 Mar
REPOSITORIES: biostudies-literature
Stem cell reports 20190214 3
It has been challenging to generate in vitro models of alveolar lung diseases, as the stable culture of alveolar type 2 (AT2) cells has been difficult. Methods of generating and expanding AT2 cells derived from induced pluripotent stem cells (iPSCs) have been established and are expected to be applicable to disease modeling. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by dysfunction of lysosome-related organelles, such as lamellar bodies (LBs), in AT2 cells. ...[more]