Ontology highlight
ABSTRACT:
SUBMITTER: Susanto E
PROVIDER: S-EPMC7443968 | biostudies-literature | 2020 Aug
REPOSITORIES: biostudies-literature
Proceedings of the National Academy of Sciences of the United States of America 20200803 33
Medulloblastoma is the most common malignant brain tumor in children. Here we describe a medulloblastoma model using Induced pluripotent stem (iPS) cell-derived human neuroepithelial stem (NES) cells generated from a Gorlin syndrome patient carrying a germline mutation in the sonic hedgehog (SHH) receptor <i>PTCH1.</i> We found that Gorlin NES cells formed tumors in mouse cerebellum mimicking human medulloblastoma. Retransplantation of tumor-isolated NES (tNES) cells resulted in accelerated tumo ...[more]