| First Author | Unachukwu U | Year | 2021 |
| Journal | iScience | Volume | 24 |
| Issue | 7 | Pages | 102684 |
| PubMed ID | 34222844 | Mgi Jnum | J:308160 |
| Mgi Id | MGI:6728163 | Doi | 10.1016/j.isci.2021.102684 |
| Citation | Unachukwu U, et al. (2021) Renal neoplasms in tuberous sclerosis mice are neurocristopathies. iScience 24(7):102684 |
| abstractText | Tuberous sclerosis (TS) is a rare disorder exhibiting multi-systemic benign neoplasms. We hypothesized the origin of TS neoplastic cells derived from the neural crest given the heterogeneous ecto-mesenchymal phenotype of the most common TS neoplasms. To test this hypothesis, we employed Cre-loxP lineage tracing of myelin protein zero (Mpz)-expressing neural crest cells (NCCs) in spontaneously developing renal tumors of Tsc2 (+/-) /Mpz(Cre)/TdT (fl/fl) reporter mice. In these mice, ectopic renal tumor onset was detected at 4 months of age increasing in volume by 16 months of age with concomitant increase in the subpopulation of tdTomato(+) NCCs from 0% to 6.45% of the total number of renal tumor cells. Our results suggest that Tsc2 (+/-) mouse renal tumors arise from domiciled proliferative progenitor cell populations of neural crest origin that co-opt tumorigenesis due to mutations in Tsc2 loci. Targeting neural crest antigenic determinants will provide a potential alternative therapeutic approach for TS pathogenesis. |
