|Application ||WB, E|
|Description||This gene encodes a protein that 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. It is also 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. Additionally, mutations in a long range enhancer located approximately 1 megabase upstream of this gene disrupt limb patterning and can result in preaxial polydactyly.|
|Immunogen||Purified recombinant fragment of human SHH expressed in E. Coli.|
|Formulation||Ascitic fluid containing 0.03% sodium azide.|
|Other Names||Sonic hedgehog protein, SHH, HHG-1, Sonic hedgehog protein N-product, Sonic hedgehog protein C-product, SHH|
|Dilution||WB~~1/500 - 1/2000|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||SHH Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Intercellular signal essential for a variety of patterning events during development: signal produced by the notochord that induces ventral cell fate in the neural tube and somites, and the polarizing signal for patterning of the anterior- posterior axis of the developing limb bud. Displays both floor plate- and motor neuron-inducing activity. The threshold concentration of N-product required for motor neuron induction is 5-fold lower than that required for floor plate induction. Activates the transcription of target genes by interacting with its receptor PTCH1 to prevent normal inhibition by PTCH1 on the constitutive signaling activity of SMO (By similarity).|
|Cellular Location||Sonic hedgehog protein C-product: Secreted, extracellular space. Note=The C-terminal peptide diffuses from the cell.|
|Tissue Location||Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues|
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1. Cancer Lett. 2010 Jan 1;287(1):44-53. 2. Oncogene. 2009 Oct 8;28(40):3513-25. 3. J Biol Chem. 2009 Nov 20;284(47):32562-71.
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