Chromosomal Instability and Molecular Defects in Induced Pluripotent Stem Cells from Nijmegen Breakage Syndrome Patients

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Chromosomal Instability and Molecular Defects in Induced Pluripotent Stem Cells from Nijmegen Breakage Syndrome Patients

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作者：Tomer Halevy¹ ; ² ; Shira Akov¹ ; ² ; Martina Bohndorf³ ; Barbara Mlody³ ; James Adjaye³ ; Nissim Benvenisty¹ ; ² ; ⁴ ; ^{nissimb@mail.huji.ac.il" class="auth_mail" title="E-mail the corresponding author} ; Michal Goldberg² ; ^{goldbergm@mail.huji.ac.il" class="auth_mail" title="E-mail the corresponding author}
刊名：Cell Reports
出版年：2016
出版时间：30 August 2016
年：2016
卷：16
期：9
页码：2499-2511
全文大小：2620 K

文摘

•: NBS-iPSC reprogramming shows selection for karyotypically normal NBS fibroblasts
•: NBS-iPSCs display delayed response to DSBs and acquire chromosomal aberrations
•: NBS-iPSCs exhibit mitotic arrest and apoptotic block
•: NBS-NPCs display downregulation of neural genes correlated with low levels of P53

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