Huwe1 ubiquitin ligase is essential to synchronize neuronal and glial differentiation in the developing cerebellum
文献类型:期刊论文
作者 | D'Arca D1; Zhao XD1; Xu WM1; Ramirez-Martinez NC1; Iavarone A[*]1,2,3; Lasorella A[*]1,2,4 |
刊名 | PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
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出版日期 | 2010 |
卷号 | 107期号:13页码:5875-5880 |
关键词 | HECT domain cell migration nervous system development tumor suppressor |
通讯作者 | ai2102@columbia.edu ; al2179@ columbia.edu |
英文摘要 | We have generated a knockout mouse strain in which the gene coding for the ubiquitin ligase Huwe1 has been inactivated in cerebellar granule neuron precursors (CGNPs) and radial glia. These mice have a high rate of postnatal lethality and profound cerebellar abnormalities. The external granule layer of the cerebellum, which contains CGNPs, is expanded and displays aberrant proliferation and impaired differentiation of the progenitor cell population. The uncontrolled proliferation of the CGNPs is associated with accumulation of the N-Myc oncoprotein, a substrate of Huwe1, and con-sequent activation of the signaling events downstream to N-Myc. Furthermore, loss of Huwe1 in Bergmann glia leads to extensive disorganization of this cell population with layering aberrations, severe granule neuron migration defects, and persistence of ectopic clusters of granule neurons in the external granule layer. Our findings uncover an unexpected role for Huwe1 in regulating Berg-mann glia differentiation and indicate that this ubiquitin ligase orchestrates the programming of the neural progenitors that give rise to neurons and glia in the cerebellum. |
收录类别 | SCI |
资助信息 | This work was supported by grants from the National Institutes of Health–National Cancer Institute to A.L. (R01CA131126) and A.I. (R01CA085628). D.D. is supported by fellowships from Provincia di Benevento/Ministero del Lavoro, Italy. |
语种 | 英语 |
公开日期 | 2014-07-07 |
源URL | [http://159.226.149.42:8088/handle/152453/7946] ![]() |
专题 | 昆明动物研究所_科研共享资源 |
作者单位 | 1.Institute for Cancer Genetics and Departments of Columbia University Medical Center, New York, NY 10032 2.Pathology,Columbia University Medical Center, New York, NY 10032 3.Neurology,Columbia University Medical Center, New York, NY 10032 4.Pediatrics, Columbia University Medical Center, New York, NY 10032 |
推荐引用方式 GB/T 7714 | D'Arca D,Zhao XD,Xu WM,et al. Huwe1 ubiquitin ligase is essential to synchronize neuronal and glial differentiation in the developing cerebellum[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA,2010,107(13):5875-5880. |
APA | D'Arca D,Zhao XD,Xu WM,Ramirez-Martinez NC,Iavarone A[*],&Lasorella A[*].(2010).Huwe1 ubiquitin ligase is essential to synchronize neuronal and glial differentiation in the developing cerebellum.PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA,107(13),5875-5880. |
MLA | D'Arca D,et al."Huwe1 ubiquitin ligase is essential to synchronize neuronal and glial differentiation in the developing cerebellum".PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 107.13(2010):5875-5880. |
入库方式: OAI收割
来源:昆明动物研究所
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