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Basic Biology and Mechanisms of Neural Ciliogenesis and the B9 Family

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Abstract

Although the discovery of cilia is one of the earliest in cell biology, the past two decades have witnessed an explosion of new insight into these enigmatic organelles. While long believed to be vestigial, cilia have recently moved into the spotlight as key players in multiple cellular processes, including brain development and homeostasis. This review focuses on the rapidly expanding basic biology of neural cilia, with special emphasis on the newly emerging B9 family of proteins. In particular, recent findings have identified a critical role for the B9 complex in a network of protein interactions that take place at the ciliary transition zone (TZ). We describe the essential role of these protein complexes in signaling cascades that require primary (nonmotile) cilia, including the sonic hedgehog pathway. Loss or dysfunction of ciliary trafficking and TZ function are linked to a number of neurologic diseases, which we propose to classify as neural ciliopathies. When taken together, the studies reviewed herein point to critical roles played by neural cilia, both in normal physiology and in disease.

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Acknowledgments

This work was supported by the Margaret E. Early Medical Research Trust (to T. T.). We are also grateful for support from the Shapell Guerin Family Foundation and the Smidt Family Foundation (to M. D.), which helped make this work possible. We thank Dr. Pasko Rakic (Yale University School of Medicine) for helpful discussion and encouragement.

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Authors and Affiliations

  1. Department of Biomedical Sciences and Regenerative Medicine Institute, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Steven Spielberg Building Room 345, Los Angeles, CA, 90048, USA

    David Gate, Rachelle Levy, Joshua J. Breunig & Terrence Town

  2. Department of Neurosurgery and Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA

    Moise Danielpour & Terrence Town

  3. Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, 90048, USA

    Terrence Town

  4. Regenerative Medicine Institute, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Steven Spielberg Building Room 361, Los Angeles, CA, 90048, USA

    Terrence Town

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  1. David Gate
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  2. Moise Danielpour
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  3. Rachelle Levy
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  4. Joshua J. Breunig
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  5. Terrence Town
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Correspondence to Joshua J. Breunig or Terrence Town.

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Gate, D., Danielpour, M., Levy, R. et al. Basic Biology and Mechanisms of Neural Ciliogenesis and the B9 Family. Mol Neurobiol 45, 564–570 (2012). https://doi.org/10.1007/s12035-012-8276-7

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