Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons

doi:10.1016/j.neuroscience.2020.09.044

科研成果详情

题名	Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons
作者	Li, Xue1,2,3,4; Yan, Meifang1,2,3,4; Guo, Zhiqiang1,2,3,4; Yan, Li 1,2,3,4; Feng, Ruru 1,2,3,4; Zhu, Hao1,2,3,4; Tu, Xiaomeng1,2,3,4; Yu, Sidney 5; Chen, Jie-Guang1,2,3,4
发表日期	2020-11-21
发表期刊	NEUROSCIENCE 影响因子和分区
语种	英语
原始文献类型	Article
关键词	SAR1B radial migration cerebral cortex axon growth secretion
其他关键词	RADIAL MIGRATION ; MUTATIONS ; PROJECTIONS ; EXPRESSION ; MATURATION ; DISORDERS ; ANDERSON ; GTPASE ; MODES
摘要	Chylomicron Retention Disease (CMRD) is a rare inherited lipid malabsorption syndrome that exhibits a recessive hypocholesterolemia in infants. CMRD has been associated with genetic mutations of SAR1B-a member of the Arf GTPase family involved in the secretory pathway from the endoplasmic reticulum to the Golgi. CMRD patients suffer from multiple neurological deficits, the etiologies of which remain unclear. In this study, we found that Sar1b protein is expressed in developing mouse neocortex. The knockdown of Sar1b does not affect the proliferation and mitotic exit of the neural progenitors but inhibits the radial migration of the newborn cortical neurons. At postnatal day 3, the neurons stalled in the white matter fail to develop axons across the midline of the corpus callosum, resulting in the loss of the neurons later on. hSAR1B(D137N), a CMRD-associated mutant of SAR1B, also impairs the positioning of the cortical neurons in the mouse brain, suggesting a dominant-negative effect by the human heterozygous mutant. The results indicate that SAR1B is crucial to radial migration and axon morphogenesis of the cortical neurons. Our study reveals a cell-autonomous action of Sar1b, which is unrelated to lipid absorption from the gut, on the development of the cerebral cortex. (C) 2020 IBRO. Published by Elsevier Ltd. All rights reserved.
资助项目	National Natural Science Foundation of China [81571096]; Wenzhou Municipal Science and Technology Bureau, China [Y20190134]
出版者	PERGAMON-ELSEVIER SCIENCE LTD
ISSN	0306-4522
EISSN	1873-7544
卷号	449 页码:228-240
DOI	10.1016/j.neuroscience.2020.09.044
页数	13
WOS类目	Neurosciences
WOS研究方向	Neurosciences & Neurology
WOS记录号	WOS:000583160000019
收录类别	SCIE
PubMed ID	33002559
通讯作者地址	[Chen, Jie-Guang]Wenzhou Med Univ, Sch Optometry & Ophthalmol, State Key Lab Visual Sci, 270 Xueyuan Rd, Wenzhou 325027, Zhejiang, Peoples R China.
引用统计
文献类型	期刊论文
条目标识符	https://kms.wmu.edu.cn/handle/3ETUA0LF/223263
专题	眼视光学院（生物医学工程学院）、附属眼视光医院
通讯作者	Chen, Jie-Guang
作者单位	1.Wenzhou Med Univ, Sch Ophthalmol & Optometry, Wenzhou 325027, Zhejiang, Peoples R China; 2.Wenzhou Med Univ, Eye Hosp, Wenzhou 325027, Zhejiang, Peoples R China; 3.State Key Lab Optometry Ophthalmol & Vis Sci, Wenzhou, Peoples R China; 4.Zhejiang Prov Key Lab Optometry & Ophthalmol, Wenzhou 325027, Zhejiang, Peoples R China; 5.Chinese Univ Hong Kong, Sch Biomed Sci, Hong Kong 999077, Peoples R China
第一作者单位	眼视光学院（生物医学工程学院）、附属眼视光医院
通讯作者单位	眼视光学院（生物医学工程学院）、附属眼视光医院
第一作者的第一单位	眼视光学院（生物医学工程学院）、附属眼视光医院
推荐引用方式 GB/T 7714	Li, Xue,Yan, Meifang,Guo, Zhiqiang,et al. Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons[J]. NEUROSCIENCE,2020,449:228-240.
APA	Li, Xue., Yan, Meifang., Guo, Zhiqiang., Yan, Li., Feng, Ruru., ... & Chen, Jie-Guang. (2020). Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons. NEUROSCIENCE, 449, 228-240.
MLA	Li, Xue,et al."Inhibition of Sar1b, the Gene Implicated in Chylomicron Retention Disease, Impairs Migration and Morphogenesis of Developing Cortical Neurons".NEUROSCIENCE 449(2020):228-240.