ZFIN ID: ZDB-PUB-131119-11
Emilin3 is required for notochord sheath integrity and interacts with Scube2 to regulate notochord-derived Hedgehog signals
Corallo, D., Schiavinato, A., Trapani, V., Moro, E., Argenton, F., and Bonaldo, P.
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:
  • Animals
  • Antigens, Surface/metabolism*
  • Body Patterning/drug effects
  • Body Patterning/genetics
  • Down-Regulation/drug effects
  • Embryo, Nonmammalian/drug effects
  • Embryo, Nonmammalian/metabolism
  • Embryo, Nonmammalian/ultrastructure
  • Extracellular Matrix Proteins/metabolism*
  • Gene Expression Regulation, Developmental/drug effects
  • Gene Knockdown Techniques
  • HEK293 Cells
  • Hedgehog Proteins/metabolism*
  • Humans
  • Membrane Glycoproteins/metabolism*
  • Mice
  • Models, Biological
  • Morpholinos/pharmacology
  • Notochord/cytology
  • Notochord/drug effects
  • Notochord/embryology
  • Notochord/metabolism*
  • Protein Binding/drug effects
  • Protein Binding/genetics
  • Signal Transduction*/drug effects
  • Signal Transduction*/genetics
  • Up-Regulation/drug effects
  • Zebrafish/embryology*
  • Zebrafish/metabolism*
  • Zebrafish Proteins/metabolism*
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.