Granulocyte-Macrophage Colony Stimulating Factor Receptor Contributes to Plexiform Neurofibroma Initiation.

ABSTRACT: Plexiform neurofibroma (PNF) is an immune cell-rich peripheral nerve sheath tumor that develops primarily in individuals with Neurofibromatosis Type 1 (NF1). Granulocyte-macrophage colony stimulating factor receptor-β (GM-CSFR-β^c) is a shared component of receptors for the cytokines GM-CSF, IL-3, and IL-5, ligands with immunomodulatory and tumor promoting roles. In the present study, we use genetically engineered mouse model of neurofibroma. We identified the expression of GM-CSFR-β^c and GM-CSFR-α on PNF cells and on macrophages and dendritic cells in the PNF, using the Nf1^f/f; DhhCre mouse model of neurofibroma formation. Genetic deletion of GM-CSFR-β^c in this model reduced the number of PNFs, which was associated with decreased numbers of tumor

SUBMITTER: Pundavela J 

PROVIDER: S-EPMC11899227 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature