GLI3

Cilia effects upon perturbation of GLI3

Cilia number / % ciliated:

No effect

Loss-of-function effect:

Shorter cilia

Ciliogenesis screen results (5 screens)

• Kim2016: Not Reported
• Wheway et al. 2015 (siRNA) [siRNA]: No effect PMID:26167766
• Breslow et al. 2018 (CRISPR) [CRISPR]: Negative Regulator (Hh signaling, casTLE effect=2.36) PMID:29459680
• Roosing et al. 2015 (siRNA) [siRNA]: No effect PMID:26595381
• Elliott et al. 2025 (CRISPRa) [CRISPRa]: Disassembly Trigger (casTLE Effect=-3.38) PMID:41160700

Phenotypes

Human ciliopathy phenotype:

polydactyly, postaxial, type A1; preaxial polydactyly of fingers

Ciliopathy associations

• Ellis-van Creveld Syndrome-like phenotypes
• Greig Cephalopolysyndactyly Syndrome
• Pallister-Hall Syndrome
• Postaxial Polydactyly

Subcellular localization

cilia, cytosol, nucleus

Functional category

• Ciliary assembly/disassembly
• Trafficking (BBSome, small GTPases, vesicular transport, ATPases)
• Actin & cytoskeleton regulation
• T cell biology
• Neurogenesis & migration
• Protein processing & maturation
• Cell migration & adhesion
• Signaling (Hedgehog, GPCRs, ion channels)
• Axon guidance & growth
• ECM & connective tissue
• Transcription regulation
• Cilia length regulation

Function

Hedgehog sig ling pathway effector. Loss of Polaris results in inefficient Gli3 processing. Inhibit Gli1- mediated transcription. The primary cilia length of Gli3∆/∆ satellite cells is shortened. Post-isolation, a comparable proportion of ciliated cells were found on freshly isolated myofibers from Gli3+/+ and Gli3∆/∆ mice. (35803939)