Laboratory Genes and Disease, Department of Dermatology, Medical University of Vienna (MUV), Vienna, 1090, Austria.
Genes, Development and Disease Group, Spanish National Cancer Research Centre (CNIO), Madrid, 28029, Spain.
Cell Res. 2020 Oct;30(10):885-901. doi: 10.1038/s41422-020-0370-1. Epub 2020 Jul 20.
Osteosarcoma (OS) is the most frequent primary malignant bone tumor in urgent need of better therapies. Using genetically modified mouse models (GEMMs), we demonstrate that Wnt signaling promotes c-Fos-induced OS formation via the actions of the collagen-modifying enzyme Loxl2. c-Fos/AP-1 directly regulates the expression of the Wnt ligands Wnt7b and Wnt9a in OS cells through promoter binding, and Wnt7b and Wnt9a in turn promote Loxl2 expression in murine and human OS cells through the transcription factors Zeb1 and Zeb2. Concordantly, inhibition of Wnt ligand secretion by inactivating the Wnt-less (Wls) gene in osteoblasts in c-Fos GEMMs either early or in a therapeutic setting reduces Loxl2 expression and progression of OS. Wls-deficient osteosarcomas proliferate less, are less mineralized and are enriched in fibroblastic cells surrounded by collagen fibers. Importantly, Loxl2 inhibition using either the pan-Lox inhibitor BAPN or a specific inducible shRNA reduces OS cell proliferation in vitro and decreases tumor growth and lung colonization in murine and human orthotopic OS transplantation models. Finally, OS development is delayed in c-Fos GEMMs treated with BAPN or with specific Loxl2 blocking antibodies. Congruently, a strong correlation between c-FOS, LOXL2 and WNT7B/WNT9A expression is observed in human OS samples, and c-FOS/LOXL2 co-expression correlates with OS aggressiveness and decreased patient survival. Therefore, therapeutic targeting of Wnt and/or Loxl2 should be considered to potentiate the inadequate current treatments for pediatric, recurrent, and metastatic OS.
骨肉瘤(OS)是最常见的原发性恶性骨肿瘤,急需更好的治疗方法。我们使用基因修饰的小鼠模型(GEMMs)证明,Wnt 信号通过胶原修饰酶 Loxl2 的作用促进 c-Fos 诱导的 OS 形成。c-Fos/AP-1 通过启动子结合直接调节 OS 细胞中 Wnt 配体 Wnt7b 和 Wnt9a 的表达,Wnt7b 和 Wnt9a 反过来通过转录因子 Zeb1 和 Zeb2 促进鼠和人 OS 细胞中 Loxl2 的表达。一致地,通过在 c-Fos GEMMs 中的成骨细胞中失活 Wnt 无(Wls)基因来抑制 Wnt 配体分泌,无论是早期还是在治疗环境中,都可以降低 Loxl2 的表达并减缓 OS 的进展。Wls 缺陷骨肉瘤增殖较少,矿化较少,富含被胶原纤维包围的成纤维细胞。重要的是,使用泛 Lox 抑制剂 BAPN 或特异性诱导性 shRNA 抑制 Loxl2 可减少体外 OS 细胞增殖,并减少鼠和人原位 OS 移植模型中的肿瘤生长和肺转移。最后,在接受 BAPN 或特异性 Loxl2 阻断抗体治疗的 c-Fos GEMMs 中,OS 发展被延迟。一致地,在人 OS 样本中观察到 c-FOS、LOXL2 和 WNT7B/WNT9A 表达之间存在很强的相关性,并且 c-FOS/LOXL2 共表达与 OS 的侵袭性和患者生存率降低相关。因此,针对 Wnt 和/或 Loxl2 的治疗靶向应该被考虑用于增强对儿科、复发性和转移性 OS 的现有治疗方法。