Essential roles of leucine-rich glioma inactivated 1 in the development of embryonic and postnatal cerebellum.

Essential roles of leucine-rich glioma inactivated 1 in the development of embryonic and postnatal cerebellum.
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富含亮氨酸的胶质瘤灭活1在胚胎和出生后小脑发育中的重要作用

DOI:
10.1038/srep07827
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发表时间:
2015-01-16
期刊:
影响因子:
4.6
通讯作者:
Shen Y
Shen Y
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Xie YJ;Zhou L;Jiang N;Zhang N;Zou N;Zhou L;Wang Y;Cowell JK;Shen Y

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Leucine-rich glioma inactivated 1 (LGI1) is a secreted protein that interacts with ADAM transmembrane proteins and its mutations are linked to human epilepsy. The function of LGI1 in CNS development remains undefined. Here, we report novel functions of LGI1 in the generation of cerebellar granule precursors (CGPs) and differentiation of radial glial cells (RGCs) in the cerebellum. A reduction in external granule layer thickness and defects in foliation were seen in embryonic and new-born LGI1 knockout (KO) mice. BrdU staining showed an inhibited proliferation of CGPs in KO embryos, which might be explained by the reduced Sonic hedgehog in embryos. In addition, the differentiation of RGCs into Bergmann glias was suppressed in KO mice. Enhanced Jagged1-Notch1 signaling in KO miceviareduced β-secretase proteolysis suggests that altered phenotype of RGCs is due to abnormal Notch1 signaling. Together, our results demonstrate that LGI1 is an essential player in the cerebellar development.
DOI: 10.1042/an20120095
发表时间: 2013-06-25
期刊: ASN neuro
影响因子: 4.7
作者:
Kegel L;Aunin E;Meijer D;Bermingham JR
通讯作者: Bermingham JR
DOI: 10.1038/ng832
发表时间: 2002-03-01
期刊: NATURE GENETICS
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发表时间: 2012-11-01
期刊: GLIA
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影响因子: 3.4
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发表时间: 2010-10
期刊: GLIA
影响因子: 6.2
作者:
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通讯作者: Bock, Hans H.