Orlova Anna, Wingelhofer Bettina, Neubauer Heidi A, Maurer Barbara, Berger-Becvar Angelika, Keserű György Miklós, Gunning Patrick T, Valent Peter, Moriggl Richard
a Institute of Animal Breeding and Genetics , University of Veterinary Medicine Vienna , Vienna , Austria.
b Ludwig Boltzmann Institute for Cancer Research , Vienna , Austria.
Expert Opin Ther Targets. 2018 Jan;22(1):45-57. doi: 10.1080/14728222.2018.1406924. Epub 2017 Nov 24.
Hematopoietic neoplasms are often driven by gain-of-function mutations of the JAK-STAT pathway together with mutations in chromatin remodeling and DNA damage control pathways. The interconnection between the JAK-STAT pathway, epigenetic regulation or DNA damage control is still poorly understood in cancer cell biology. Areas covered: Here, we focus on a broader description of mutational insights into myeloproliferative neoplasms and peripheral T-cell leukemia and lymphomas, since sequencing efforts have identified similar combinations of driver mutations in these diseases covering different lineages. We summarize how these pathways might be interconnected in normal or cancer cells, which have lost differentiation capacity and drive oncogene transcription. Expert opinion: Due to similarities in driver mutations including epigenetic enzymes, JAK-STAT pathway activation and mutated checkpoint control through TP53, we hypothesize that similar therapeutic approaches could be of benefit in these diseases. We give an overview of how driver mutations in these malignancies contribute to hematopoietic cancer initiation or progression, and how these pathways can be targeted with currently available tools.
造血系统肿瘤通常由JAK-STAT信号通路的功能获得性突变以及染色质重塑和DNA损伤控制通路的突变驱动。在癌细胞生物学中,JAK-STAT信号通路、表观遗传调控或DNA损伤控制之间的相互联系仍知之甚少。涵盖领域:在此,我们重点更广泛地描述对骨髓增殖性肿瘤以及外周T细胞白血病和淋巴瘤的突变见解,因为测序工作已在这些涵盖不同谱系的疾病中鉴定出相似的驱动突变组合。我们总结了这些信号通路在正常细胞或已丧失分化能力并驱动癌基因转录的癌细胞中可能如何相互连接。专家观点:由于包括表观遗传酶、JAK-STAT信号通路激活以及通过TP53的突变检查点控制在内的驱动突变存在相似性,我们推测相似的治疗方法可能对这些疾病有益。我们概述了这些恶性肿瘤中的驱动突变如何导致造血系统癌症的起始或进展,以及如何利用现有工具靶向这些信号通路。