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Author Notes:

Correspondence: tcaspar@emory.edu

We are grateful to Alyssa Long and Sarah Suciu for sequencing potential CRISPR founders, and to members of the Caspary lab for discussion and manuscript comments.

Disclosures: No competing interests declared.


Research Funding:

This work was supported by funding from NIH grants R01NS090029 and R35GM122549 to TC and R35GM122568 to RAK. EDG was supported by NIH training grant T32NS096050 and a diversity supplement to R01NS090029.

Further support came from the Emory Integrated Mouse Transgenic and Gene Targeting Core, which is subsidized by the Emory University School of Medicine and with support by the Georgia Clinical and Translational Science Alliance of the National Institutes of Health under Award Number UL1TR002378 as well as the Emory University Integrated Cellular Imaging Microscopy Core of the Emory Neuroscience NINDS Core Facilities grant, P30NS055077.

National Institute of Neurological Disorders and Stroke R01NS090029 to Tamara Caspary.

National Institute of General Medical Sciences R35GM122549 to Tamara Caspary.

National Institute of General Medical Sciences R35GM122568 to Richard A Kahn.

National Institute of Neurological Disorders and Stroke T32NS096050 to Eduardo D Gigante.

National Institute of Neurological Disorders and Stroke F31 NS106755 to Eduardo D Gigante.


  • Science & Technology
  • Life Sciences & Biomedicine
  • Biology
  • Life Sciences & Biomedicine - Other Topics
  • Intraflagellar transport
  • Joubert syndrome
  • Protein trafficiking
  • Small gtpases
  • Floor plate
  • Gene
  • Transduction
  • Regeneration
  • Ciliogenesis
  • Drosophila

ARL13B regulates Sonic hedgehog signaling from outside primary cilia

Journal Title:



Volume 9


Type of Work:

Article | Final Publisher PDF


ARL13B is a regulatory GTPase highly enriched in cilia. Complete loss of Arl13b disrupts cilia architecture, protein trafficking and Sonic hedgehog signaling. To determine whether ARL13B is required within cilia, we knocked in a cilia-excluded variant of ARL13B (V358A) and showed it retains all known biochemical function. We found that ARL13BV358A protein was expressed but could not be detected in cilia, even when retrograde ciliary transport was blocked. We showed Arl13bV358A/V358A mice are viable and fertile with normal Shh signal transduction. However, in contrast to wild type cilia, Arl13bV358A/V358A cells displayed short cilia and lacked ciliary ARL3 and INPP5E. These data indicate that ARL13B’s role within cilia can be uncoupled from its function outside of cilia. Furthermore, these data imply that the cilia defects upon complete absence of ARL13B do not underlie the alterations in Shh transduction, which is unexpected given the requirement of cilia for Shh transduction.

Copyright information:

© 2020, Gigante et al.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/).
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