期刊
HUMAN MOLECULAR GENETICS
卷 20, 期 6, 页码 1061-1073出版社
OXFORD UNIV PRESS
DOI: 10.1093/hmg/ddq549
关键词
-
资金
- National Basic Research Program of China (NBRP 973 project) [2006CB806700, 2007CB512205]
- National Hi-Tech Research and Development Program of China (863 project) [2007AA022101]
- Chinese Ministry of Education
- National Nature Science Foundation of China [30971650, 30872825]
- Shanghai Subject Chief Scientist Program [09XD1400900]
- Shanghai Pujiang Program [09PJ1402400]
- NIH [EY015851, EY03040]
- Research to Prevent Blindness
Nuclear movement relative to cell bodies is a fundamental process during certain aspects of mammalian retinal development. During the generation of photoreceptor cells in the cell division cycle, the nuclei of progenitors oscillate between the apical and basal surfaces of the neuroblastic layer (NBL). This process is termed interkinetic nuclear migration (INM). Furthermore, newly formed photoreceptor cells migrate and form the outer nuclear layer (ONL). In the current study, we demonstrated that a KASH domain-containing protein, Syne-2/Nesprin-2, as well as SUN domain-containing proteins, SUN1 and SUN2, play critical roles during INM and photoreceptor cell migration in the mouse retina. A deletion mutation of Syne-2/Nesprin-2 or double mutations of Sun1 and Sun2 caused severe reduction of the thickness of the ONL, mislocalization of photoreceptor nuclei and profound electrophysiological dysfunction of the retina characterized by a reduction of a-and b-wave amplitudes. We also provide evidence that Syne-2/Nesprin-2 forms complexes with either SUN1 or SUN2 at the nuclear envelope to connect the nucleus with dynein/dynactin and kinesin molecular motors during the nuclear migrations in the retina. These key retinal developmental signaling results will advance our understanding of the mechanism of nuclear migration in the mammalian retina.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据