Sonic Hedgehog (SHH) Rabbit Polyclonal Antibody

CAT#: TA363692

SHH Antibody - middle region

Product Images

Host: Rabbit Target Name: SHH Sample Tissue: Human HepG2 Whole Cell lysates Antibody Dilution: 1ug/ml

Specifications

Product Data

• Applications: WB
• Reactivities: Human
• Host: Rabbit
• Clonality: Polyclonal
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human SHH
• Specificity: Expected reactivity: Human
• Formulation: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Concentration: lot specific
• Purification: Affinity purified
• Conjugation: Unconjugated
• Storage: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Predicted Protein Size: 50 kDa
• Gene Name: sonic hedgehog
• Database Link:
  NP_000184.1
  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: HHG-1; HHG1; HLP3; HPE3; MCOPCB5; SMMCI; TPT; TPTPS