BUBR1 deficiency results in abnormal megakaryopoiesis
BUBR1 deficiency results in abnormal megakaryopoiesis
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DOI：
10.1182/blood-2003-06-2158
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发表时间：
2004-02-15
期刊：
影响因子：
20.3
通讯作者：
Dai, W
中科院分区：
文献类型：
--
作者：
Wang, Q;Liu, TY;Dai, W
通过产生BUBR 1突变小鼠来检查BIJBR 1的生理功能，BIJBR 1是纺锤体检查点的关键组分。BUBR 1（-/-）胚胎在子宫内由于广泛的凋亡而不能存活超过8.5天。而BUBR 1（+/-）囊胚在体外生长相对正常，BUBR 1（-/-）囊胚表现出受损的增殖和萎缩。成年BUBR 1（+/-）小鼠表现为脾肿大，BUBR 1（+/-）小鼠中异常巨核细胞生成与外周血血小板显著增加无关，这至少部分是由于产生前血小板的巨核细胞形成缺陷所致。总之，这些结果表明BUBR 1对早期胚胎发育和正常造血至关重要。(C)2004年，美国血液学会。
The physiologic function of BIJBR1, a key component of the spindle checkpoint, was examined by generating BUBR1-mutant mice. BUBR1(-/-) embryos failed to survive beyond day 8.5 in utero as a result of extensive apoptosis. Whereas BUBR1(+/-) blastocysts grew relatively normally in vitro, BUBR1(-/-) blastocysts exhibited impaired proliferation and atrophied. Adult BUBR1(+/-) mice manifested splenomegaly and abnormal megakaryo-esis in BUBR1(+/-) mice was not correlated with a significant increase in platelets in peripheral blood, which was at least partly due to a defect in the formation of proplatele-producing megakaryocytes. Together, these results indicate that BUBR1 is essential for early embryonic development and normal hematopoiesis. (C) 2004 by The American Society of Hematology.