Hedgehog (Hh) proteins are widely distributed throughout the animal kingdom and play important regulatory roles in various development processes. The three known mammalian Hh proteins, Sonic (Shh), Desert (Dhh) and Indian (Ihh) are structurally related and share a high degree of amino acid sequence identity (e.g. Shh and Ihh are 93 % identical). Although, Hh proteins have unique expression patterns and distinct biological roles within their respective regions of secretion, they signal through the same Patched (Ptc)/Smoothened (Smo) receptor complex, activate the same intracellular signaling pathway, and can substitute for each other in experimental systems. The mature biologically active form of Hh molecules is produced by autocatalytic cleavage of their precursor proteins and corresponds to approximately the N-terminal half of the precursor molecule. Recombinant human Shh is a 20.6 kDa protein consisting of 174 amino acid residues, which correspond to the active N-terminal portion of the Shh precursor.
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