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Sonic Hedgehog (SHH)

Sonic Hedgehog is a highly conserved protein that plays an important role in embryonic development. It is expressed in neural tissue, the gut, and areas of limb development and promotes differentiation and growth in a tissue-specific manner. SHH is synthesized as a 45-kDa precursor protein that is cleaved to generate the active 19-kDa N-terminus. SHH interacts with the Patched and Smoothened transmembrane receptors, leading to the activation of GLI family transcription factors. Disruption of any part of this pathway during embryogenesis is associated with birth defects ranging from mild to severe. In adults, abnormal activation of the SHH pathway has been implicated in several forms of cancer.