The role of primary cilia in neuronal function.
The role of primary cilia in neuronal function.
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DOI：
10.1016/j.nbd.2009.12.022
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发表时间：
2010-05
影响因子：
6.1
通讯作者：
Gleeson JG
中科院分区：
文献类型：
--
作者：
Lee JH;Gleeson JG
“纤毛病”是一组新定义的疾病，其特征在于细胞初级纤毛的结构或功能缺陷。患有这些疾病的患者表现出过度表达的纤维囊性肾病、视网膜盲、多指（趾）畸形、肥胖和脑发育不全以及神经认知障碍。Joubert综合征是一种由小脑蚓部发育不全、眼神经失用症、间歇性换气过度和智力迟钝所定义的纤毛病。最近的证据表明，初级纤毛在介导一系列细胞外信号事件，如形态发生、有丝分裂原、稳态和极性信号中起重要作用。基于纤毛病和纤毛介导的信号通路的临床特征，数据支持初级纤毛在调节神经发生、细胞极性、轴突引导和可能的成人神经元功能中的作用。
The “ciliopathies” are a newly defined group of disorders characterized by defects in the structure or function of the cellular primary cilium. Patients with these disorders display variably expressive fibrocystic renal disease, retinal blindness, polydactyly, obesity, and brain dysgenesis as well as neurocognitive impairments. Joubert syndrome is a ciliopathy defined by cerebellar vermis hypoplasia, oculomotor apraxia, intermittent hyperventilation, and mental retardation. Recent evidence suggests important roles for the primary cilium in mediating a host of extracellular signaling events such as morphogen, mitogen, homeostatic and polarity signals. Based upon the clinical features of ciliopathies and cilia mediated signaling pathways, the data support a role for the primary cilium in modulating neurogenesis, cell polarity, axonal guidance and possibly adult neuronal function.
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影响因子：
3.9
作者：
Brancati, Francesco;Iannicelli, Miriam;Travaglini, Lorena;Mazzotta, Annalisa;Bertini, Enrico;Boltshauser, Eugen;D'Arrigo, Stefano;Emma, Francesco;Fazzi, Elisa;Gallizzi, Romina;Gentile, Mattia;Loncarevic, Damir;Mejaski-Bosnjak, Vlatka;Pantaleoni, Chiara;Rigoli, Luciana;Salpietro, Carmelo D.;Signorini, Sabrina;Stringini, Gilda Rita;Verloes, Alain;Zabloka, Dominika;Dallapiccola, Bruno;Gleeson, Joseph G.;Valente, Enza Maria
通讯作者：
Valente, Enza Maria
影响因子：
3.3
作者：
Händel, M;Schulz, S;Höllt, V
通讯作者：
Höllt, V
影响因子：
64.8
作者：
Andersen, JS;Wilkinson, CJ;Mann, M
通讯作者：
Mann, M
DOI：
10.1073/pnas.0804558105
发表时间：
2008-09-02
影响因子：
11.1
作者：
Breunig, Joshua J.;Sarkisian, Matthew R.;Town, Terrence
通讯作者：
Town, Terrence
影响因子：
9.8
作者：
Dixon-Salazar, T;Silhavy, JL;Gleeson, JG
通讯作者：
Gleeson, JG