Emilin3 is required for notochord sheath integrity and interacts with Scube2 to regulate notochord-derived Hedgehog signals
- Authors
- Corallo, D., Schiavinato, A., Trapani, V., Moro, E., Argenton, F., and Bonaldo, P.
- ID
- ZDB-PUB-131119-11
- Date
- 2013
- Source
- Development (Cambridge, England) 140(22): 4594-4601 (Journal)
- Registered Authors
- Argenton, Francesco, Corallo, Diana, Moro, Enrico
- Keywords
- extracellular matrix, EMILIN, notochord, hedgehog, scube2, zebrafish
- MeSH Terms
-
- Antigens, Surface/metabolism*
- Notochord/cytology
- Notochord/drug effects
- Notochord/embryology
- Notochord/metabolism*
- Down-Regulation/drug effects
- Mice
- Extracellular Matrix Proteins/metabolism*
- Models, Biological
- Protein Binding/drug effects
- Protein Binding/genetics
- Gene Expression Regulation, Developmental/drug effects
- Humans
- Zebrafish/embryology*
- Zebrafish/metabolism*
- Animals
- Body Patterning/drug effects
- Body Patterning/genetics
- HEK293 Cells
- Embryo, Nonmammalian/drug effects
- Embryo, Nonmammalian/metabolism
- Embryo, Nonmammalian/ultrastructure
- Membrane Glycoproteins/metabolism*
- Signal Transduction*/drug effects
- Signal Transduction*/genetics
- Up-Regulation/drug effects
- Zebrafish Proteins/metabolism*
- Morpholinos/pharmacology
- Hedgehog Proteins/metabolism*
- Gene Knockdown Techniques
- PubMed
- 24131633 Full text @ Development
The notochord is a transient and essential structure that provides both mechanical and signaling cues to the developing vertebrate embryo. In teleosts, the notochord is composed of a core of large vacuolated cells and an outer layer of cells that secrete the notochord sheath. In this work, we have identified the extracellular matrix glycoprotein Emilin3 as a novel essential component of the zebrafish notochord sheath. The development of the notochord sheath is impaired in Emilin3 knockdown embryos. The patterning activity of the notochord is also affected by Emilin3, as revealed by the increase of Hedgehog (Hh) signaling in Emilin3-depleted embryos and the decreased Hh signaling in embryos overexpressing Emilin3 in the notochord. In vitro and in vivo experiments indicate that Emilin3 modulates the availability of Hh ligands by interacting with the permissive factor Scube2 in the notochord sheath. Overall, this study reveals a new role for an EMILIN protein and reinforces the concept that structure and function of the notochord are strictly linked.