Shh, Recombinant, Human (Sonic HedgeHog)

Members of the Hedgehog (Hh) family are highly conserved proteins which are widely represented throughout the animal kingdom. 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). The biologically active form of Hh molecules is obtained by autocatalytic cleavage of their precursor proteins and corresponds to approximately the N-terminal one half of the precursor molecule. Although Hh proteins have unique expression patterns and distinct biological roles within their respective regions of secretion, they use the same signaling pathway and can substitute for each other in experimental systems.\n\nSonic Hedgehog, which is expressed only during embryogenesis, 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 disorder in which the developing forebrain fails to correctly separate into right and left hemispheres. HPE is manifested by facial deformities. In addition, it is thought that mutations in this gene or in its signalling pathway may be responsible for VACTERL syndrome, which is characterized by vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal dysplasia, cardiac anomalies, and limb abnormalities.\n\nA DNA sequence encoding amino acid residues Cys 24

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SPECIFICATIONS

Catalog Number

S1012-96

Size

25ug

Form

Supplied as a lyophilized powder from PBS, BSA. Reconstitute with sterile PBS, (same/more than) 0.1% HSA or BSA to make a final concentration of (same/more than) 50ug/ml.

Purity

~ 97% by SDS-PAGE, silver stain Endotoxin: (same/less than)EU/ug

References

1. Carpenter, D. et al., 1998, Proc. Natl. Acad. Sci. USA 95:13630. 2. Perrimon, N., 1995, Cell 80:517. 3. Weed, M. et al., 1997, Matrix Biol. 16:53. 4. Mullor, J. et al., 2002, Trends Cell Biol. 12:562. 5. Ingham, P. and A. McMahon, 2001, Genes & Dev. 15:3059.