Development of 3D culture models of plexiform neurofibroma and initial application for phenotypic characterization and drug screening.

ABSTRACT: Plexiform neurofibromas (PNs), which may be present at birth in up to half of children with type 1 neurofibromatosis (NF1), can cause serious loss of function, such as quadriparesis, and can undergo malignant transformation. Surgery is the first line treatment although the invasive nature of these tumors often prevents complete resection. Recent clinical trials have shown promising success for some drugs, notably selumetinib, an inhibitor of MAP kinase kinase (MEK). We have developed three-dimensional (3D) cell culture models of immortalized cells from NF1 PNs and of control Schwann cells (SCs) that we believe mimic more closely the in vivo condition than conventional two-dimensional (2D) cell culture. Our goal is to facilitate pre-clinical identification of potential targeted therapeutics

SUBMITTER: Kraniak JM 

PROVIDER: S-EPMC6863155 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature