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Leukaemia and the quality control of ribosome assembly

发布日期:2017-12-05 作者: 来源:bv伟德国际体育 点击:

讲座题目Leukaemia and the quality control of ribosome assembly

讲座人 Alan Warren教授

时间2017125日(星期二)上午10:00

地点:长安校区 格物楼3201会议室

讲座内容简介

The synthesis of new ribosomes is a fundamental conserved process in all cells. Ribosomes are pre-assembled in the nucleus and exported to the cytoplasm, where they acquire functionality through a series of final maturation steps that include the formation of the catalytic centre, recruitment of the last remaining ribosomal proteins and the removal of inhibitory assembly factors. A major surprise in the last few years has been the identification of mutations in key ribosome assembly factors in both inherited and sporadic forms of leukaemia. In particular, biallelic mutations in the SBDS gene cause Shwachman-Diamond syndrome, a recessive bone marrow failure disorder associated with significant predisposition to acute myeloid leukaemia. By studying the pathogenesis of this rare disease, we have uncovered an elegant mechanism that couples the final step in maturation of the large ribosomal subunit to a quality control assessment of the structural integrity of the active sites of the ribosome.

讲座人简介:

Alan Warren博士,现任剑桥大学血液系教授,剑桥大学医学研究所和干细胞研究所研究员。1988年于剑桥大学MRC Laboratory of Molecular Biology(LMB)获得博士学位。2003年获聘剑桥大学血液系教授。2005年当选为英国医学科学院院士。Alan Warren教授研究工作的重点是通过阐明真核生物核糖体组装的基本机制以研究由核糖体缺陷引发的干细胞失调及癌症。他发现了白血病唯一相关蛋白Lmo-2 是造血作用必须的。他主持的研究工作在Nat Struct Mol Biol, Nature Genetic, Blood等一流刊物发表论文近百篇。2016年因为在分子生物学和生物技术领域的成就获得欧洲生化协会国际学术成就奖(FEBS National Lecture Award)。