This gene encodes a member of the hedgehog family. The
hedgehog gene family encodes signaling molecules that play an
important role in regulating morphogenesis. This protein is
predicted to be made as a precursor that is autocatalytically
cleaved; the N-terminal portion is soluble and contains the
signalling activity while the C-terminal portion is involved in
precursor processing. More importantly, the C-terminal product
covalently attaches a cholesterol moiety to the N-terminal product,
restricting the N-terminal product to the cell surface and
preventing it from freely diffusing throughout the organism.
Defects in this protein have been associated with partial gonadal
dysgenesis (PGD) accompanied by minifascicular polyneuropathy. This
protein may be involved in both male gonadal differentiation and
perineurial development.
