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Harry Potter. Popular Features. New Releases. Description Cilia are tiny microtubule-based organelles projecting from the plasma membrane of practically all cells in the body. In the past 10 years a flurry of research has indicated a crucial role of this long-neglected organelle in the development and function of the central nervous system. A common theme of these studies is the critical dependency of signal transduction of the Sonic hedgehog, and more recently, Wnt signaling pathways upon cilia to regulate fate decisions and morphogenesis.

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Currently such barriers are not known in spines. Also, do components of ciliary transport machinery eg. Genotype-phenotype correlations in ciliopathies with CNS disruption? We do not have a good understanding as to why ciliopathies such as Joubert Syndrome JBTS can present with large symptomatic variation across different patient cohorts. Mutations in the same gene can give rise to a strikingly different phenotype. Discussion centered around the importance of investigating allele-specific effects on the JBTS phenotype, and how cell-specific mechanisms of JBTS gene function can influence the disease phenotype.

Where are cilia localized on neurons? The location of cilia in different cell types in different parts of the brain are unknown.


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  5. Initial electron microscopy reconstructions of the brain appear to demonstrate tremendous heterogeneity in the sites and structures of neuronal cilia. Establishing this issue is important since ciliary signaling from different cellular locations may influence neuronal properties via different pathways. How are signals from cilia transmitted to distant neuronal sites?

    A hallmark of many neurons is the presence of long processes with synapses present at sites as distant as several meters from the cell body. How are signals from the cilia transmitted to these distant sites? Possibilities are via second messenger molecules generated by ciliary signaling, or longer-term effects mediated by transcriptional mechanisms initiated by signals from the cilia. What role might ciliary signaling play in the maintenance of neuronal activity?

    Maintaining the proper balance between excitatory and inhibitory synaptic input is crucial for neuronal circuit function. An intriguing possibility is that cilia integrate disparate signals from many sources in a neuronal circuit, and then use those signals to adjust excitatory or inhibitory synaptic strength. This model is highly speculative but intriguing preliminary results suggest it should be investigated further. The seminar successfully interested participating neuroscientists in the roles of cilia in the brain, and informed cilia biologists about brain complexity and function.

    The seminar also allowed two groups of scientists who may not otherwise communicate to meet and interact extensively. The presentations and discussions highlighted the many fascinating and complex questions in the field, and led to the establishment of at least two collaborations to begin to address some of these issues.

    We thank the Fondation for their support of this very successful and interesting seminar and look forward to learning more about the functions of cilia in the brain in the future.