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Reconstitution: Centrifuge the vial briefly, before opening to bring the contents to the bottom. Reconstitute the lyophilized protein in sterile, 20 mM Citrate Buffer pH 5.0 or 10 mM Acetic Acid to a concentration of 0.1-0.5 mg/mL to regain full activity. Apportion the reconstituted protein into working aliquots and store at ≤-20°C. Make any further dilutions of there constituted protein in medium or buffered solution containing carrier protein, such as PBS with 0.1% BSA.
Storage: Store the lyophilized protein at 2-8°C, preferably desiccated. Upon reconstitution, apportion into working aliquots and store at ≤-20°C (not in a frost-free freezer). Avoid repeated freeze-thaw cycles.
Sonic Hedgehog (SHH), 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: hemimelic extra toes; HHG-1; SHH; Shh unprocessed N-terminal signaling and C-terminal autoprocessing domains; ShhNC; short digits; Sonic hedgehog protein
Gene Aliases: 9530036O11Rik; Dsh; Hhg1; Hx; Hxl3; M100081; Shh
UniProt ID: (Mouse) Q62226
Entrez Gene ID: (Mouse) 20423
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