Article

Molecular Neurobiology

, Volume 45, Issue 3, pp 564-570

Basic Biology and Mechanisms of Neural Ciliogenesis and the B9 Family

  • David GateAffiliated withDepartment of Biomedical Sciences and Regenerative Medicine Institute, Cedars-Sinai Medical Center
  • , Moise DanielpourAffiliated withDepartment of Neurosurgery and Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center
  • , Rachelle LevyAffiliated withDepartment of Biomedical Sciences and Regenerative Medicine Institute, Cedars-Sinai Medical Center
  • , Joshua J. BreunigAffiliated withDepartment of Biomedical Sciences and Regenerative Medicine Institute, Cedars-Sinai Medical Center Email author 
  • , Terrence TownAffiliated withDepartment of Biomedical Sciences and Regenerative Medicine Institute, Cedars-Sinai Medical CenterDepartment of Neurosurgery and Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical CenterDepartment of Medicine, David Geffen School of Medicine, University of CaliforniaRegenerative Medicine Institute, Cedars-Sinai Medical Center Email author 

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

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.

Keywords

Primary cilia Neural ciliogenesis Neural ciliopathy B9-C2 family Ciliary signaling Stem cell Progenitor