|Application ||WB, IHC|
|Reactivity||Human, Mouse, Rat|
|Calculated MW||53947 Da|
|Other Names||Suppressor of fused homolog, SUFUH, SUFU|
|Target/Specificity||A synthetic peptide corresponding to peptides on human Su(Fu) was used as an immunogen.|
|Format||50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||SUFU Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Negative regulator in the hedgehog signaling pathway. Down-regulates GLI1-mediated transactivation of target genes. Part of a corepressor complex that acts on DNA-bound GLI1. May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome. Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein. Negative regulator of beta-catenin signaling. Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full length form of GLI3 (GLI3FL). GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state. Without the Hh signal, the SUFU- GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R. When Hh signaling is initiated, SUFU dissociates from GLI3FL and the latter translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Required for the proper formation of hair follicles and the control of epidermal differentiation (By similarity).|
|Cellular Location||Cytoplasm. Nucleus.|
|Tissue Location||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 fetal brain, lung and kidney|
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Provided below are standard protocols that you may find useful for product applications.
The Hedgehog (Hh) signaling pathway has conserved roles in development of species ranging from Drosophila to humans. Human supressor of fused HSu(Fu) acts as a negative regulator in the hedgehog signaling (SHH) pathway by down-regulating GLI1-mediated transactivation of target genes (1). HSu(fu) was found to repress activity of the zinc-finger transcription factor Gli, which mediates Hedgehog signaling in vertebrates, and to physically interact with Gli, Gli2 and Gli3 (2). The sonic hedgehog signaling pathway directs the embryonic development of diverse organisms and is disrupted in a variety of malignancies. Several of mutations encode truncated proteins that are unable to export the GLI transcription factor from nucleus to cytoplasm, resulting in the activation of SHH signaling. SUFU is a newly identified tumor-suppressor gene that predisposes individuals to medulloblastoma by modulating the SHH signaling pathway through a newly identified mechanism (3).
1. Kogerman P, et al. Nat Cell Biol 1(5):312-9, 1999.
2. Stone DM, et al. J Cell Sci 112(Pt23):4437-48, 1999.
3. Taylor MD, et al. Nat Genet 31(3):306-10, 2002.
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