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Human Shh protein (active)

Cat No. GTX48138-pro

Application

Functional Assay

Species

Human
Package
25 μg ($369)
PRODUCT

Summary

Recombinant E. coli derived Human Sonic HedgeHog is a 20.0 kDa protein consisting of 176 amino acid residues, including an N-terminal Ile-Val-Ile sequence substituted for the natural occurring chemically modified Cys residue

APPLICATION

Application Note

Determined by its ability to induce alkaline phosphatase production by C3H/10T1/2 (CCL-226) cells. The expected ED₅₀ for this effect is 0.8-1.0 μg/ml.

PROPERTIES

Form

Lyophilized powder

Buffer

Batch dependent (please contact us for details)

Storage

Store at -20ºC or below. After reconstitution, keep as concentrated solution. Avoid freeze-thaw cycles.

Region/Sequence

IVIGPGRGFGKRRHPKKLTPLAYKQFIPNVAEKTLGASGRYEGKISRNSERFKELTPNYNPDIIFKDEENTGADRLMTQRCKDKLNALAISVMNQWPGVKLRVTEGWDEDGHHSEESLHYEGRALDITTSDRDRSKYGMLARLAVEAGFDWVYYESKAHIHCSVKAENSVAAKSGG

Expression System

E. Coli

Purity

> 98%

Endotoxin

< 1 EU/μg

Conjugation

Unconjugated

Note

For laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.

TARGET

Synonyms

sonic hedgehog signaling molecule , HHG1 , HLP3 , HPE3 , MCOPCB5 , SMMCI , ShhNC , TPT , TPTPS

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. [provided by RefSeq, Jul 2008]

Database

Research Area

REFERENCE

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REVIEW

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