Renal neoplasms in tuberous sclerosis mice are neurocristopathies.
Renal neoplasms in tuberous sclerosis mice are neurocristopathies.
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DOI：
10.1016/j.isci.2021.102684
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发表时间：
2021-07-23
期刊：
影响因子：
5.8
通讯作者：
D'Armiento J
中科院分区：
文献类型：
--
作者：
Unachukwu U;Shiomi T;Goldklang M;Chada K;D'Armiento J
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)/TdTfl/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. Renal and hepatic tumors in Tsc2+/− mice comprise of neural crest cells (NCCs) NCC proliferation increased with Tsc2+/− mice tumor volume and multiplicity Multipotent Tsc2+/− NCCs induce tumors in ectodermally derived and non-neural tissues Pharmacologically targeting NCCs offers an alternate treatment for tuberous sclerosis Molecular physiology; Stem cells research; Cancer;
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影响因子：
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