Sonic Hedgehog (SHH) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC
Recommend Dilution	IHC: 50-100 Positive control: Human breast cancer Predicted cell location: Cytoplasm, ExtraCellular space
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide corresponding to a region derived from 448-462 amino acids of Human sonic hedgehog
Formulation	PBS pH7.3, 0.05% NaN3, 50% glycerol
Concentration	lot specific
Purification	Antigen affinity purification
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Gene Name	sonic hedgehog
Database Link	NP_000184 Entrez Gene 20423 MouseEntrez Gene 29499 RatEntrez 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	HHG1; HLP3; HPE3; MCOPCB5; SMMCI; TPT; TPTPS
Reference Data
Protein Families	Druggable Genome, ES Cell Differentiation/IPS, Secreted Protein, Transmembrane
Protein Pathways	Basal cell carcinoma, Hedgehog signaling pathway, Pathways in cancer

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