Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations.

Roumiantsev S; Krause DS; Neumann CA; Dimitri CA; Asiedu F; Cross NC; Van Etten RA

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Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations.

机译：由8p11易位引起的ZNF198-FGFR1和BCR-FGFR1融合基因诱导的明显的干细胞骨髓增生性/ T淋巴瘤综合征。

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8p11 myeloproliferative syndrome (EMS) is a hematopoietic stem cell disorder characterized by myeloid hyperplasia and non-Hodgkin's lymphoma with chromosomal translocations fusing several genes, most commonly ZNF198, to fibroblast growth factor receptor-1 (FGFR1). However, patients with BCR-FGFR1 fusion present with typical chronic myeloid leukemia (CML). We demonstrate that ZNF198-FGFR1 induces EMS-like disease in mice, with myeloproliferation and T lymphoma arising from common multipotential progenitors. Mutation of FGFR1 Tyr766 attenuates both myeloid and lymphoid diseases, identifying phospholipase C-gamma1 as a downstream effector. Bcr-FGFR1 binds Grb2 via Bcr Tyr177 and induces CML-like leukemia in mice, whereas Bcr-FGFR1/Y177F lacks Grb2 binding and causes EMS-like disease. These results implicate different signaling pathways originating from both kinase and fusion partner in the pathogenesis of CML and EMS.

机译：8p11骨髓增生异常综合征（EMS）是一种造血干细胞疾病，其特征是髓样增生和非霍奇金淋巴瘤，染色体易位，将几种基因（最常见的ZNF198）融合到成纤维细胞生长因子受体1（FGFR1）中。然而，具有BCR-FGFR1融合的患者表现出典型的慢性粒细胞白血病（CML）。我们证明ZNF198-FGFR1诱导小鼠骨髓增生和T淋巴瘤由常见的多能祖细胞引起的EMS样疾病。 FGFR1 Tyr766的突变减弱了髓样和淋巴样疾病，将磷脂酶C-γ1鉴定为下游效应子。 Bcr-FGFR1通过Bcr Tyr177结合Grb2并诱导小鼠CML样白血病，而Bcr-FGFR1 / Y177F缺乏Grb2结合并引起EMS样疾病。这些结果暗示了在CML和EMS的发病机理中源自激酶和融合伴侣的不同信号通路。

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《Cancer Cell》 |2004年第3期|共12页
作者
Roumiantsev S; Krause DS; Neumann CA; Dimitri CA; Asiedu F; Cross NC; Van Etten RA;
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正文语种 eng
中图分类肿瘤学;
关键词
fibroblast growth factor receptor 1; Electron Microscopy; BCR Gene; International Normalized Ratio; 国际标准化比;

机译：fibroblast growth factor receptor 1;Electron Microscopy;BCR Gene;International Normalized Ratio;国际标准化比;

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1. Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations. [J] . Roumiantsev S, Krause DS, Neumann CA, Cancer Cell . 2004,第3期

机译：由8p11易位引起的ZNF198-FGFR1和BCR-FGFR1融合基因诱导的明显的干细胞骨髓增生性/ T淋巴瘤综合征。
2. Favorable outcome of allogeneic hematopoietic cell transplantation for 8p11 myeloproliferative syndrome associated with BCR-FGFR1 gene fusion [J] . DolanM., CiocA., CrossN.C.P., Pediatric blood & cancer . 2012,第1期

机译：同种异体造血细胞移植与BCR-FGFR1基因融合相关的8p11骨髓增生综合征的良好结果
3. Allogeneic hematopoietic stem cell transplantation for 8p11 myeloproliferative syndrome with BCR-FGFR1 gene rearrangement: a case report and literature review [J] . Konishi Yoshinobu, Kondo Tadakazu, Nakao Kensuke, Bone marrow transplantation . 2019,第2期

机译：同种异体造血干细胞移植8P11肌酚综合征，BCR-FGFR1基因重排 - 案例报告与文献综述
4. Classification of White Blood Cell Abnormalities for Early Detection of Myeloproliferative Neoplasms Syndrome Using Backpropagation [C] . Zilvanhisna Emka Fitri, Arizal Mujibtamala Nanda Imronl International Conference on Electronics, Biomedical Engineering, and Health Informatics . 2020

机译：白细胞异常对早期检测髓原肿瘤综合征使用反向衰减的分类
5. Generation of Non-Integrated Induced Pluripotent Stem Cells from Hutchinson Gilford Progeria Syndrome Patient Fibroblasts and Differentiation to Mesodermal Lineage [D] . Kanherkar, Riya Rajan. 2018

机译：Hutchinson Gilford早衰综合症患者成纤维细胞非整合诱导多能干细胞的生成及向中胚层谱系的分化
6. Ponatinib as targeted therapy for FGFR1 fusions associated with the 8p11 myeloproliferative syndrome [O] . Andrew Chase, Catherine Bryant, Joannah Score, 2013

机译：Ponatinib作为与8p11骨髓增生综合征相关的FGFR1融合的靶向治疗
7. Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations [O] . Roumiantsev Sergei, Krause Daniela S, Neumann Carola A, 2004

机译：由8p11易位引起的ZNF198-FGFR1和BCR-FGFR1融合基因诱导的明显干细胞骨髓增生性/ T淋巴瘤综合征

Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations.

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