SUFU encodes a component of the Sonic hedgehog (SHH; MIM 600725)/Patched (PTCH; MIM 601309) signaling pathway. Mutations in genes encoding components of this pathway are deleterious for normal development and are associated with cancer-predisposing syndromes (e.g., HPE3, MIM 142945; BCNS, MIM 109400; and GCPS, MIM 175700)Zimmern P,et al.(2010) Neurourol Urodyn.29(5):715-8.Wang C,et al. (2010) Development. 137(12):2001-9. Rabasseda X.et al.(2010) Drug News Perspect.23(4):273-5.
Host
Rabbit
Immunogen
Peptide sequence around phosphorylation site of Serine 342 (A-P-S(p)-R-K) derived from Human Sufu.
SUFU encodes a component of the Sonic hedgehog (SHH; MIM 600725)/Patched (PTCH; MIM 601309) signaling pathway. Mutations in genes encoding components of this pathway are deleterious for normal development and are associated with cancer-predisposing syndromes (e.g., HPE3, MIM 142945; BCNS, MIM 109400; and GCPS, MIM 175700)
Zimmern P,et al.(2010) Neurourol Urodyn.29(5):715-8. Wang C,et al. (2010) Development. 137(12):2001-9. Rabasseda X.et al.(2010) Drug News Perspect.23(4):273-5.
Species
Homo Sapiens (Human)
Specificity
The antibody detects endogenous level of Sufu protein only when phosphorylated at serine 342.
Negative regulator in the hedgehog signaling pathway. Down-regulates GLI1-mediated transactivation of target genes
Pathway
Hedgehog signaling pathway
Protein Families
SUFU family
Tissue Specificity
Ubiquitous in adult tissues. Detected in osteoblasts of the perichondrium in the developing limb of 12-week old embryos. Isoform 1 is detected in fetal brain, lung, kidney and testis. Isoform 2 is detected in fetal testis, and at much lower levels in feta
Buffer
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format
liquid
Purification
Antibodies were produced by immunizing rabbits with synthetic peptide and KLH conjugates. Antibodies were purified by affinity-chromatography using epitope-specific peptide.
Purity
Antibodies were produced by immunizing rabbits with synthetic peptide and KLH conjugates. Antibodies were purified by affinity-chromatography using epitope-specific peptide.
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.