The full-length unprocessed hedgehog protein is an active signaling molecule

J Biol Chem. 2010 Jan 22;285(4):2562-8. doi: 10.1074/jbc.M109.078626. Epub 2009 Nov 17.

Abstract

The hedgehog (HH) family of ligands plays an important instructional role in metazoan development. HH proteins are initially produced as approximately 45-kDa full-length proteins, which undergo an intramolecular cleavage to generate an amino-terminal product that subsequently becomes cholesterol-modified (HH-Np). It is well accepted that this cholesterol-modified amino-terminal cleavage product is responsible for all HH-dependent signaling events. Contrary to this model we show here that full-length forms of HH proteins are able to traffic to the plasma membrane and participate directly in cell-cell signaling, both in vitro and in vivo. We were also able to rescue a Drosophila eye-specific hh loss of function phenotype by expressing a full-length form of hh that cannot be processed into HH-Np. These results suggest that in some physiological contexts full-length HH proteins may participate directly in HH signaling and that this novel activity of full-length HH may be evolutionarily conserved.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Communication / physiology
  • Chick Embryo
  • Chickens
  • Drosophila
  • Drosophila Proteins / chemistry
  • Drosophila Proteins / genetics
  • Drosophila Proteins / metabolism
  • Evolution, Molecular
  • Gene Expression Regulation, Developmental*
  • Hedgehog Proteins* / chemistry
  • Hedgehog Proteins* / genetics
  • Hedgehog Proteins* / metabolism
  • Holoprosencephaly / genetics
  • Holoprosencephaly / physiopathology
  • Humans
  • Mutagenesis, Site-Directed
  • Neural Tube / embryology
  • Neural Tube / physiology
  • Patched Receptors
  • Phenotype
  • Protein Structure, Tertiary
  • Protein Transport / physiology
  • Rabbits
  • Receptors, Cell Surface / metabolism
  • Signal Transduction / physiology*
  • Structure-Activity Relationship

Substances

  • Drosophila Proteins
  • Hedgehog Proteins
  • Patched Receptors
  • Receptors, Cell Surface
  • SHH protein, human
  • hh protein, Drosophila