Sonic Hedgehog (SHH) Mouse Monoclonal Antibody [Clone ID: 8G3]

Specifications

Product Data
Clone Name	8G3
Applications	ELISA, IHC, WB
Recommend Dilution	ELISA: 1/10000. Western Blot: 1/500-1/2000. Immunuohistochemistry on Paraffin Sections: 1/200.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Purified recombinant fragment of human SHH expressed in E. Coli.
Specificity	This antibody recognizes Human Sonic Hedgehog / SHH. Other species not tested.
Formulation	State: Ascites Preservative: 0.03% Sodium Azide
Conjugation	Unconjugated
Storage Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Predicted Protein Size	49.6 kDa
Gene Name	sonic hedgehog
Database Link	Entrez Gene 6469 Human UniProt ID Q15465
Background	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.
Synonyms	Sonic hedgehog protein, HHG-1
Reference Data

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