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Specifications

Clone Host Grade Applications Monoclonal Mouse E B IH FC

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.
Catalog #	030623
Applications	Western Blot: 1:500-1:2000
	Immunohistochemistry: 1:200-1:1000
	Flow Cytometry: 1:200-1:400
	ELISA: 1:10000
Storage and Stability	May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone Type	Monoclonal
Isotype	IgG1
Clone No	13B701
Host	Mouse
Source	Human
Concentration	Not determined
Form	Purified antibody in PBS with 0.05% sodium azide and 0.5% protein stabilizer
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Purity	Purified
Immunogen	Purified recombinant fragment of human SHH (AA: 26-161) expressed in E. coli.
Specificity	Human, Mouse, Monkey
Important Note	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Alternate names	TPT; HHG1; HLP3; HPE3; SMMCI; TPTPS; MCOPCB5