Program in Molecular Medicine.
Department of Molecular, Cell and Cancer Biology.
J Clin Invest. 2018 Jan 2;128(1):294-308. doi: 10.1172/JCI92513. Epub 2017 Dec 4.
Oncogenomic studies indicate that copy number variation (CNV) alters genes involved in tumor progression; however, identification of specific driver genes affected by CNV has been difficult, as these rearrangements are often contained in large chromosomal intervals among several bystander genes. Here, we addressed this problem and identified a CNV-targeted oncogene by performing comparative oncogenomics of human and zebrafish melanomas. We determined that the gene encoding growth differentiation factor 6 (GDF6), which is the ligand for the BMP family, is recurrently amplified and transcriptionally upregulated in melanoma. GDF6-induced BMP signaling maintained a trunk neural crest gene signature in melanomas. Additionally, GDF6 repressed the melanocyte differentiation gene MITF and the proapoptotic factor SOX9, thereby preventing differentiation, inhibiting cell death, and promoting tumor growth. GDF6 was specifically expressed in melanomas but not melanocytes. Moreover, GDF6 expression levels in melanomas were inversely correlated with patient survival. Our study has identified a fundamental role for GDF6 and BMP signaling in governing an embryonic cell gene signature to promote melanoma progression, thus providing potential opportunities for targeted therapy to treat GDF6-positive cancers.
肿瘤基因组学研究表明,拷贝数变异 (CNV) 改变了肿瘤进展相关的基因;然而,由于这些重排通常包含在几个旁观者基因之间的大染色体间隔内,因此很难确定受 CNV 影响的特定驱动基因。在这里,我们通过对人类和斑马鱼黑色素瘤的比较肿瘤基因组学研究解决了这个问题,并鉴定了一个 CNV 靶向的致癌基因。我们确定,编码生长分化因子 6 (GDF6) 的基因,即 BMP 家族的配体,在黑色素瘤中经常扩增和转录上调。GDF6 诱导的 BMP 信号在黑色素瘤中维持着躯干神经嵴基因特征。此外,GDF6 抑制黑素细胞分化基因 MITF 和促凋亡因子 SOX9,从而阻止分化、抑制细胞死亡并促进肿瘤生长。GDF6 特异性表达于黑色素瘤中,但不表达于黑素细胞中。此外,黑色素瘤中 GDF6 的表达水平与患者的生存呈负相关。我们的研究表明,GDF6 和 BMP 信号在调控胚胎细胞基因特征以促进黑色素瘤进展方面发挥了基本作用,从而为针对 GDF6 阳性癌症的靶向治疗提供了潜在机会。