Product Type: Antibody
Descritpion: 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.
Research Areas: Cancer, Epigenetics and Nuclear Signaling, Metabolism, Developmental biology, Stem cells
Recommended Dilutions: IHC 1:50-1:200
Size: 200 uL
Gene Accession: NP_000184
Immunogen: Synthetic peptide of human SHH
Formulation: PBS with 0.05% sodium azide, 50% glycerol, PH7.3
Purification: Affinity purification
Tissue Specificity/Positive Control: Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues.
Cellular Localization: Cell membrane. The N-product either remains associated with lipid rafts at the cell surface, or forms freely diffusible active multimers with its hydrophobic lipid-modified N- and C-termini buried inside and Secreted>extracellular space. The C-terminal peptide diffuses from the cell.
Storage: Store at -20C. Avoid freeze / thaw cycles.
Product Link: https://www.elabscience.com/p-shh_polyclonal_antibody-32080.html
Manual Link: https://www.elabscience.com/viewpdf-32080-elabscience-E-AB-12636.PDF