© 2003 BMJ Publishing Group & Association of Clinical Pathologists
EDITORIAL
Sonic hedgehog
Sonic hedgehog
1 Division of Child Health, School of Human Development, University of Nottingham NG7 1EN, UK
2 Division of Clinical Genetics, City Hospital, Hucknall Road, Nottingham NG5 1PB, UK
Correspondence to:
Correspondence to:
Dr H S Heussler, Division of Child Health, School of Human Development, University of Nottingham NG7 1EN, UK;
honey.Heussler@nottingham.ac.uk
Implications for human development
Keywords: Sonic hedgehog; patterning; embryogenesis; oncogenic transformation; gut malformations; fundic gland differentiation
| The first 150 words of the full text of this article appear below. |
The hedgehog (hh) gene is one of the segment polarity genes that regulate segmental and imaginal disc patterning in the fruit fly, Drosophila melanogaster. Unlike drosophila and other invertebrates, which only have a single hh gene, vertebrates have a family of genes that are homologous to the hh gene. Mammals have three genes with homology to the hh gene. These comprise Sonic hedgehog (Shh), Indian hedgehog (Ihh), and Desert hedgehog (Dhh).1 All hedgehog genes encode signalling molecules that are involved in short and long range patterning processes during embryogenesis.
Like all hedgehog proteins Shh protein also undergoes molecular processing in the endoplasmic reticulum. This involves cleavage of its signal peptide, followed by autocatalytic cleavage of the hedgehog protein precursor into a 19 kDa N-terminal domain (Shh-N) and a 25 kDa C-terminal domain (Shh-C). The signalling activity of hedgehog proteins resides in Shh-N.2 Shh-C has intramolecular cholesterol transferase
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