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Essential roles of leucine-rich glioma inactivated 1 in the development of embryonic and postnatal cerebellum.
Xie, Ya-Jun; Zhou, Liang; Jiang, Nanwei; Zhang, Nan; Zou, Na; Zhou, Lin; Wang, Yin; Cowell, John K; Shen, Ying.
Afiliación
  • Xie YJ; Department of Neurobiology, Key Laboratory of Medical Neurobiology of the Ministry of Health, Zhejiang Province Key Laboratory of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China.
  • Zhou L; Department of Neurobiology, Key Laboratory of Medical Neurobiology of the Ministry of Health, Zhejiang Province Key Laboratory of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China.
  • Jiang N; Zhejiang Provincial Key Laboratory of Pathophysiology, Department of Physiology and Pharmacology, Ningbo University School of Medicine, Ningbo, China.
  • Zhang N; Ningxia Key Laboratory of Cerebrocranial Diseases, Ningxia Medical University, Yinchuan, China.
  • Zou N; Department of Neurobiology, Key Laboratory of Medical Neurobiology of the Ministry of Health, Zhejiang Province Key Laboratory of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China.
  • Zhou L; Department of Neurobiology, Key Laboratory of Medical Neurobiology of the Ministry of Health, Zhejiang Province Key Laboratory of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China.
  • Wang Y; Ningxia Key Laboratory of Cerebrocranial Diseases, Ningxia Medical University, Yinchuan, China.
  • Cowell JK; Georgia Regents University, Cancer Center, Augusta, GA, USA.
  • Shen Y; Department of Neurobiology, Key Laboratory of Medical Neurobiology of the Ministry of Health, Zhejiang Province Key Laboratory of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China.
Sci Rep ; 5: 7827, 2015 Jan 16.
Article en En | MEDLINE | ID: mdl-25591666
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 mice via reduced ß-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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Proteínas / Cerebelo / Embrión de Mamíferos Límite: Animals Idioma: En Revista: Sci Rep Año: 2015 Tipo del documento: Article País de afiliación: China Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Proteínas / Cerebelo / Embrión de Mamíferos Límite: Animals Idioma: En Revista: Sci Rep Año: 2015 Tipo del documento: Article País de afiliación: China Pais de publicación: Reino Unido