NP_000184.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
Observed: 51 kDa Predicted: 50 kDa
NCBI Official Full Name
sonic hedgehog protein isoform 1 preproprotein
NCBI Official Synonym Full Names
sonic hedgehog
NCBI Official Synonym Symbols
TPT; HHG1; HLP3; HPE3; SMMCI; ShhNC; TPTPS; MCOPCB5 [Similar Products]
NCBI Protein Information
sonic hedgehog protein
UniProt Protein Name
Sonic hedgehog protein
UniProt Synonym Protein Names
HHG-1; Shh unprocessed N-terminal signaling and C-terminal autoprocessing domains1 PublicationManual assertion based on opinion iniRef.16"Scube2 enhances proteolytic Shh processing from the surface of Shh-producing cells."Jakobs P., Exner S., Schuermann S., Pickhinke U., Bandari S., Ortmann C., Kupich S., Schulz P., Hansen U., Seidler D.G., Grobe K.J. Cell Sci. 127:1726-1737(2014) [PubMed] [Europe PMC] [Abstract]Cited for: PTM, INTERACTION WITH SCUBE2, SUBUNIT, SUBCELLULAR LOCATION, FUNCTION, CAUTION.
UniProt Synonym Gene Names
SHH; ShhNC1 PublicationManual assertion based on opinion iniRef.16; ShhN; ShhNp1 PublicationManual assertion based on opinion iniRef.16 [Similar Products]
NCBI Summary for Shh
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]
UniProt Comments for Shh
Sonic hedgehog protein: The C-terminal part of the sonic hedgehog protein precursor displays an autoproteolysis and a cholesterol transferase activity (). Both activities result in the cleavage of the full-length protein into two parts (ShhN and ShhC) followed by the covalent attachment of a cholesterol moiety to the C-terminal of the newly generated ShhN (). Both activities occur in the reticulum endoplasmic (). Once cleaved, ShhC is degraded in the endoplasmic reticulum ().
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Products associated with anti-Shh antibody
Pathways associated with anti-Shh antibody
Diseases associated with anti-Shh antibody
Organs/Tissues associated with anti-Shh antibody
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