Sonic Hedgehog activates prostaglandin signaling to stabilize primary cilium length

J Cell Biol. 2024 Sep 2;223(9):e202306002. doi: 10.1083/jcb.202306002. Epub 2024 Jun 10.

Abstract

Sonic Hedgehog (SHH) is a driver of embryonic patterning that, when corrupted, triggers developmental disorders and cancers. SHH effector responses are organized through primary cilia (PC) that grow and retract with the cell cycle and in response to extracellular cues. Disruption of PC homeostasis corrupts SHH regulation, placing significant pressure on the pathway to maintain ciliary fitness. Mechanisms by which ciliary robustness is ensured in SHH-stimulated cells are not yet known. Herein, we reveal a crosstalk circuit induced by SHH activation of Phospholipase A2α that drives ciliary E-type prostanoid receptor 4 (EP4) signaling to ensure PC function and stabilize ciliary length. We demonstrate that blockade of SHH-EP4 crosstalk destabilizes PC cyclic AMP (cAMP) equilibrium, slows ciliary transport, reduces ciliary length, and attenuates SHH pathway induction. Accordingly, Ep4-/- mice display shortened neuroepithelial PC and altered SHH-dependent neuronal cell fate specification. Thus, SHH initiates coordination between distinct ciliary receptors to maintain PC function and length homeostasis for robust downstream signaling.

MeSH terms

  • Animals
  • Cilia* / metabolism
  • Cyclic AMP / metabolism
  • Hedgehog Proteins* / genetics
  • Hedgehog Proteins* / metabolism
  • Mice
  • Mice, Knockout
  • Prostaglandins* / metabolism
  • Receptors, Prostaglandin E, EP4 Subtype / genetics
  • Receptors, Prostaglandin E, EP4 Subtype / metabolism
  • Signal Transduction*

Substances

  • Cyclic AMP
  • Hedgehog Proteins
  • Prostaglandins
  • Receptors, Prostaglandin E, EP4 Subtype
  • Shh protein, mouse