[HTML][HTML] Stromal Lkb1 deficiency leads to gastrointestinal tumorigenesis involving the IL-11–JAK/STAT3 pathway

S Ollila, E Domènech-Moreno… - The Journal of …, 2018 - Am Soc Clin Investig
S Ollila, E Domènech-Moreno, K Laajanen, IPL Wong, S Tripathi, N Pentinmikko, Y Gao
The Journal of clinical investigation, 2018Am Soc Clin Investig
Germline mutations in the gene encoding tumor suppressor kinase LKB1 lead to
gastrointestinal tumorigenesis in Peutz-Jeghers syndrome (PJS) patients and mouse
models; however, the cell types and signaling pathways underlying tumor formation are
unknown. Here, we demonstrated that mesenchymal progenitor-or stromal fibroblast–
specific deletion of Lkb1 results in fully penetrant polyposis in mice. Lineage tracing and
immunohistochemical analyses revealed clonal expansion of Lkb1-deficient myofibroblast …
Germline mutations in the gene encoding tumor suppressor kinase LKB1 lead to gastrointestinal tumorigenesis in Peutz-Jeghers syndrome (PJS) patients and mouse models; however, the cell types and signaling pathways underlying tumor formation are unknown. Here, we demonstrated that mesenchymal progenitor- or stromal fibroblast–specific deletion of Lkb1 results in fully penetrant polyposis in mice. Lineage tracing and immunohistochemical analyses revealed clonal expansion of Lkb1-deficient myofibroblast-like cell foci in the tumor stroma. Loss of Lkb1 in stromal cells was associated with induction of an inflammatory program including IL-11 production and activation of the JAK/STAT3 pathway in tumor epithelia concomitant with proliferation. Importantly, treatment of LKB1-defcient mice with the JAK1/2 inhibitor ruxolitinib dramatically decreased polyposis. These data indicate that IL-11–mediated induction of JAK/STAT3 is critical in gastrointestinal tumorigenesis following Lkb1 mutations and suggest that targeting this pathway has therapeutic potential in Peutz-Jeghers syndrome.
The Journal of Clinical Investigation