Sonic Hedgehog Homolog

Expressed only during embryogenesis by human SHH Gene (Hedgehog Family), 462-aa 50-kDa (precursor) Sonic Hedgehog Homolog is the notochord inductive signal in ventral neural tube and somite patterning and the polarizing signal for anterior-posterior limb axis patterning. The C-terminal domain displays precursor processing autoproteolytic activity that generates the soluble signaling N-portion. With transferase activity, the C-portion covalently attaches cholesterol to the new N-product C-terminus, restricting it to the cell surface and regulating distribution of protein activity throughout the embryo. SHH binds to PTC receptor, releasing constitutive SMO repression and activating target gene transcription. Defects in SHH cause HPE3. (NCI Thesaurus)

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