scholarly journals Photoreceptor Sensory Cilium and Associated Disorders

10.5772/48387 ◽  
2012 ◽  
Author(s):  
Linjing Li ◽  
Ozge Yildiz ◽  
Manisha Anand ◽  
Hemant Kh
Keyword(s):  
Sensory Cilium

scholarly journals Defining the Layers of a Sensory Cilium with STORM and Cryo-Electron Nanoscopies

2017 ◽  
Author(s):  
Michael A. Robichaux ◽  
Valencia L. Potter ◽  
Zhixian Zhang ◽  
Feng He ◽  
Michael F. Schmid ◽  
...  
Keyword(s):  
Primary Cilia ◽  
Electron Tomography ◽  
Super Resolution ◽  
Intraflagellar Transport ◽  
Structural Features ◽  
Animal Cells ◽  
Ciliary Membrane ◽  
Acetylated Tubulin ◽  
Sensory Cilium ◽  
Syntaxin 3

ABSTRACTPrimary cilia are cylindrical organelles extending from the surface of most animal cells that have been implicated in a host of signaling and sensory functions. Genetic defects in their component molecules, known as “ciliopathies” give rise to devastating symptoms, ranging from defective development, to kidney disease, to progressive blindness. The detailed structures of these organelles and the true functions of proteins encoded by ciliopathy genes are poorly understood because of the small size of cilia and the limitations of conventional microscopic techniques. We describe the combination of cryo-electron tomography, enhanced by sub-tomogram averaging, with super-resolution stochastic reconstruction microscopy (STORM) to define substructures and subdomains within the light-sensing rod sensory cilium of the mammalian retina. Longitudinal and radial domains are demarcated by structural features such as the axoneme and its connections to the ciliary membrane, and are correlated with molecular markers of these compartments, including Ca2+-binding protein centrin-2 in the lumen of the axoneme, acetylated tubulin forming the axoneme, the glycocalyx extending outward from the surface of the plasma membrane, and molecular residents of the space between axoneme and ciliary membrane, including Arl13B, intraflagellar transport proteins, BBS5, and syntaxin-3. Within this framework we document that deficiencies in the ciliopathy proteins BBS2, BBS7 and BBS9 lead to inappropriate accumulation of proteins in rod outer segments while largely preserving their sub-domain localization within the connecting cilium region, but alter the distribution of syntaxin-3 clusters.

Diffusion of a soluble protein, photoactivatable GFP, through a sensory cilium

2010 ◽  
Vol 188 (5) ◽  
pp. i11-i11
Author(s):  
Peter D. Calvert ◽  
William E. Schiesser ◽  
Edward N. Pugh
Keyword(s):  
Soluble Protein ◽  
Sensory Cilium ◽  
Photoactivatable Gfp

scholarly journals Three-Dimensional Architecture of the Rod Sensory Cilium and Its Disruption in Retinal Neurodegeneration

Cell ◽  
2012 ◽  
Vol 151 (5) ◽  
pp. 1029-1041 ◽  
Author(s):  
Jared C. Gilliam ◽  
Juan T. Chang ◽  
Ivette M. Sandoval ◽  
Youwen Zhang ◽  
Tiansen Li ◽  
...  
Keyword(s):  
Three Dimensional ◽  
Sensory Cilium ◽  
Retinal Neurodegeneration

scholarly journals Worms With a Single Functional Sensory Cilium Generate Proper Neuron-Specific Behavioral Output

Genetics ◽  
2009 ◽  
Vol 183 (2) ◽  
pp. 595-605 ◽  
Author(s):  
Gabriele Senti ◽  
Marina Ezcurra ◽  
Jana Löbner ◽  
William R. Schafer ◽  
Peter Swoboda
Keyword(s):  
Sensory Cilium
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