比利時研究人員日前發(fā)現(xiàn),，兩種特殊蛋白質(zhì)的缺乏會使大腦腦液流動異常，從而可能引發(fā)腦積水等腦部疾病,。
保護人腦的腦液通常在大腦細小的孔洞里流動,，一日更新數(shù)次，一旦流動不暢就會引起大腦孔洞的擴張,，顱壓升高,，從而造成腦積水。
比利時魯汶天主教大學研究人員2日發(fā)布公告說,，腦液的流動是由大腦“活動纖毛”推動的,，他們發(fā)現(xiàn)蛋白質(zhì)Celsr2和Celsr3的缺乏會造成大腦“活動纖毛”工作不正常，引起腦液流動異常,，導致顱壓升高,，損害神經(jīng)細胞，最終造成腦積水和腦死亡,。
研究人員說,，此項研究成果有助于了解神經(jīng)系統(tǒng)的工作機理，從而使醫(yī)學專家更好地研究人腦某些缺損的成因,。(生物谷Bioon.com)
生物谷推薦原文出處：
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.
