摘要
BCOR是一种表观遗传因子,广泛分布于人体中,常与眼面心牙(oculocardiofaciodental,OFCD)综合征相关联。学者在血液系统相关疾病如骨髓增生异常综合征、急性髓系白血病、再生障碍性贫血等疾病中发现BCOR基因突变。BCOR参与了胚胎干细胞、骨髓间充质干细胞的发育,同时具有调控造血的功能,维持骨髓造血生态位的稳定。BCOR突变与血液系统相关疾病的发病进程、预后分层显著相关,BCOR突变对再生障碍性贫血而言是良性突变,但对骨髓增生异常综合征与急性髓系白血病而言却是恶性突变,可为临床个性化选择治疗方案以及预后分层的判断提供更多依据,具有较大临床意义与应用价值。故本文就BCOR在骨髓造血生态位中的作用关系,以及BCOR突变对血液系统疾病的病程进展、预后分层等方面的国内外研究进展作一系统综述。
BCOR is a kind of epigenetic factors, which are widely distributed in the human body, and often causes eye tooth face centered(oculocardiofaciodental OFCD) syndrom, BCOR mutations are found in myelodysplastic syndrome, acute myeloid leukemia, aplastic anemia and other diseases related diseases of the blood. Studies have found that BCOR participates in the development of embryonic stem cells and bone marrow mesenchymal stem cells, and has the function of regulating hematopoietic and maintaining the stability of bone marrow hematopoietic niche. According to a new study, BCOR mutations associated with blood system diseases were significantly associated with the infection process and prognosis of layered, BCOR mutation for aplastic anemia is a benign mutation, but is a malignant mutation for myelodysplastic syndrome and acute myeloid leukemia. It can be chosen for clinical individualized treatment plan and prognosis of layered judgment. It has great clinical significance and application value. In this paper, the role of BCOR in the hematopoietic niche of bone marrow, as well as BCOR mutation on the progression of blood system diseases,prognosis stratification and other aspects of the domestic and foreign research progress are systematically reviewed.
作者
谷梦宇
闫赛
于孙婉琪
牛丁忍
王金环
GU Mengyu;YAN Sai;YU Sunwanqi;NIU Dingren;WANG Jinhuan(Graduate School,Heilongjiang University of Traditional Chinese Medicine,Harbin 150006,China;Clinical Medical College,Anhui University of Traditional Chinese Medicine,Hefei 230012,China;Department of Hematology,the First Affiliated Hospital of Heilongjiang University of Traditional Chinese Medicine,Harbin 150040,China)
出处
《生命的化学》
CAS
2022年第6期1117-1123,共7页
Chemistry of Life
基金
国家自然科学基金项目(81763923)
黑龙江省博士后科研启动资金项目(LBH-Q14153)
黑龙江中医药大学校科研基金科技创新团队项目(2019TD03)。
