
This gene encodes a protein that is instrumental in patterning the early embryo. It has been implicated as the key inductive signal in patterning of the ventral neural tube| the anterior-posterior limb axis| and the ventral somites. Of three human proteins showing sequence and functional similarity to the sonic hedgehog protein of Drosophila| this protein is the most similar. The protein is 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 developing embryo. Defects in this protein or in its signalling pathway are a cause of holoprosencephaly (HPE)| a d
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