期刊
ISCIENCE
卷 24, 期 7, 页码 -出版社
CELL PRESS
DOI: 10.1016/j.isci.2021.102684
关键词
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资金
- Center for LAM and Rare Lung Diseases at Columbia University
- NIH [RO1-HL086936, KO8 (HL126071)]
- Congressionally Directed Medical Research Programs (CDMRP) grant [TS170057]
The study found that the pathogenesis of tuberous sclerosis originates from neural crest cell populations, which play a crucial role in renal tumors in Tsc2(+/-) mice. Targeting neural crest antigenic determinants may offer a potential alternative therapeutic approach for TS pathogenesis.
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.
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