BUBR1 deficiency results in abnormal megakaryopoiesis

被引：144

作者：

Wang, Q

Liu, TY

Fang, YQ

Xie, SQ

Huang, X

Mahmood, R

Ramaswamy, G

Sakamoto, KM

Darzynkiewicz, Z

Xu, M

Dai, W

机构：

[1] New York Med Coll, Dept Med, Div Mol Carcinogenesis, Brander Canc Inst, Valhalla, NY 10595 USA

[2] New York Med Coll, Dept Pathol, Valhalla, NY 10595 USA

[3] Albert Einstein Coll Med, Core Facil Histopathol, Bronx, NY 10467 USA

[4] Univ Calif Los Angeles, Mattel Childrens Hosp, Div Hematol Oncol,Mol Biol Inst,David Geffen Sch, Dept Pathol & Lab Med,Jonsson Comprehens Canc Ctr, Los Angeles, CA USA

[5] Univ Cincinnati, Coll Med, Dept Cell Biol, Cincinnati, OH USA

来源：

BLOOD | 2004年 / 103卷 / 04期

关键词：

D O I：

10.1182/blood-2003-06-2158

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1278 / 1285

页数：8

共 35 条

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