比利时研究人员日前发现，两种特殊蛋白质的缺乏会使大脑脑液流动异常，从而可能引发脑积水等脑部疾病。
保护人脑的脑液通常在大脑细小的孔洞里流动，一日更新数次，一旦流动不畅就会引起大脑孔洞的扩张，颅压升高，从而造成脑积水。
比利时鲁汶天主教大学研究人员2日发布公告说，脑液的流动是由大脑“活动纤毛”推动的，他们发现蛋白质Celsr2和Celsr3的缺乏会造成大脑“活动纤毛”工作不正常，引起脑液流动异常，导致颅压升高，损害神经细胞，最终造成脑积水和脑死亡。
研究人员说，此项研究成果有助于了解神经系统的工作机理，从而使医学专家更好地研究人脑某些缺损的成因。(生物谷Bioon.com)
生物谷推荐原文出处：
Nature Neuroscience 13, 700–707 (2010) doi:10.1038/nn.2555
Lack of cadherins Celsr2 and Celsr3 impairs ependymal ciliogenesis, leading to fatal hydrocephalus
Fadel Tissir, Yibo Qu, Mireille Montcouquiol, Libing Zhou, Kouji Komatsu, Dongbo Shi, Toshihiko Fujimori, Jason Labeau, Donatienne Tyteca, Pierre Courtoy, Yves Poumay, Tadashi Uemura & Andre M Goffinet
Ependymal cells form the epithelial lining of cerebral ventricles. Their apical surface is covered by cilia that beat in a coordinated fashion to facilitate circulation of the cerebrospinal fluid (CSF). The genetic factors that govern the development and function of ependymal cilia remain poorly understood. We found that the planar cell polarity cadherins Celsr2 and Celsr3 control these processes. In Celsr2-deficient mice, the development and planar organization of ependymal cilia are compromised, leading to defective CSF dynamics and hydrocephalus. In Celsr2 and Celsr3 double mutant ependyma, ciliogenesis is markedly impaired, resulting in lethal hydrocephalus. The membrane distribution of Vangl2 and Fzd3, two key planar cell polarity proteins, was disturbed in Celsr2 mutants, and even more so in Celsr2 and Celsr3 double mutants. Our findings suggest that planar cell polarity signaling is involved in ependymal cilia development and in the pathophysiology of hydrocephalus, with possible implications in other ciliopathies.
