The small GTPase RSG1 controls a final step in primary cilia initiation
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Updated: Jun 26, 2026

RhoC GTPase Activation Assay
Published on: August 22, 2010
Stephanie O Agbu1,2, Yinwen Liang1, Aimin Liu3
1Developmental Biology Program, Sloan Kettering Institute, Memorial Sloan Kettering Cancer Center, New York, NY.
Abstract:
Primary cilia, which are essential for normal development and tissue homeostasis, are extensions of the mother centriole, but the mechanisms that remodel the centriole to promote cilia initiation are poorly understood. Here we show that mouse embryos that lack the small guanosine triphosphatase RSG1 die at embryonic day 12.5, with developmental abnormalities characteristic of decreased cilia-dependent Hedgehog signaling. Rsg1 mutant embryos have fewer primary cilia than wild-type embryos, but the cilia that form are of normal length and traffic Hedgehog pathway proteins within the cilium correctly. Rsg1 mother centrioles recruit proteins required for cilia initiation and dock onto ciliary vesicles, but axonemal microtubules fail to elongate normally. RSG1 localizes to the mother centriole in a process that depends on tau tubulin kinase 2 (TTBK2), the CPLANE complex protein Inturned (INTU), and its own GTPase activity. The data suggest a specific role for RSG1 in the final maturation of the mother centriole and ciliary vesicle that allows extension of the ciliary axoneme.
Insights
The small GTPase RSG1 is crucial for primary cilia formation during embryonic development. Loss of RSG1 impairs cilia initiation and leads to developmental defects by affecting mother centriole maturation.
Area of Science:
- Cell Biology
- Developmental Biology
- Molecular Biology
Background:
- Primary cilia are vital for development and tissue homeostasis.
- Centriole remodeling mechanisms for cilia initiation are not fully understood.
Purpose of the Study:
- Investigate the role of the small GTPase RSG1 in primary cilia formation.
- Elucidate the mechanisms by which RSG1 influences centriole maturation and cilia initiation.
Main Methods:
- Analysis of mouse embryos lacking RSG1 (Rsg1 mutant).
- Assessment of primary cilia number, length, and Hedgehog pathway protein trafficking.
- Localization studies of RSG1 and associated proteins at the mother centriole.
Main Results:
- Rsg1 mutant embryos exhibit developmental abnormalities and die by embryonic day 12.5.
- Fewer primary cilia form in Rsg1 mutants, with normal length and protein trafficking.
- RSG1 is essential for mother centriole recruitment of cilia initiation proteins and docking with ciliary vesicles, but not for axonemal microtubule elongation.
Conclusions:
- RSG1 plays a critical role in the final maturation of the mother centriole and ciliary vesicles.
- This maturation step is necessary for the proper extension of the ciliary axoneme.
- RSG1 is a key regulator of cilia-dependent signaling pathways essential for embryonic development.
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