SUFU (C54G2) Rabbit mAb

• 产品编号：CST-2520S      品牌：CST       原厂货号：2520S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

此SUFU (C54G2) Rabbit mAb兔单抗识别内源性的总SUFU 蛋白。此单克隆抗体是通过一种合成的肽段去免疫动物产生。这种合成的肽段与人SUFU蛋白邻近Leu458氨基酸残基序列一致。

SUFU (Fused的抑制因子)在果蝇属中被确定为Fused（Fu）激酶的抑制因子，SUFU在胚胎形成中为Hedgehog信号通路所必需（1）。SUFU通过调控转录因子Gli和Ci的定位来抑制Hedgehog信号通路（2,3）。在果蝇中SUFU可能正向调控Hedgehog信号，而Hedgehog的信号依赖于SUFU蛋白水平和Hedgehog信号强度（4）。SUFU可能起肿瘤抑制因子作用，SUFU杂合子丢失和失活与横纹肌肉瘤以及成神经管细胞瘤有关（5,6）。SUFU缺失的小鼠导致胚胎致死，但杂合子显示基底细胞痣综合征发育缺陷特点，这种不正常发育途径归结于配体非依赖的Hh信号通路激活（7）。GSK-3β结合并磷酸化SUFU在体外，其他信息预测GSK-3β 通过SUFU的修饰正向调控Hedgehog信号通路（8）。

1. Pham, A. et al. (1995) Genetics 140, 587-598.
2. Barnfield, P.C. et al. (2005) Differentiation 73, 397-405.
3. Méthot, N. and Basler, K. (2000) Development 127, 4001-4010.
4. Dussillol-Godar, F. et al. (2006) Dev. Biol. 291, 53-66.
5. Tostar, U. et al. (2006) J. Pathol. 208, 17-25.
6. Taylor, M.D. et al. (2002) Nat. Genet. 31, 306-310.
7. Svärd, J. et al. (2006) Dev. Cell. 10, 187-197.
8. Takenaka, K. et al. (2007) Biochem. Biophys. Res. Commun. 353, 501-508.

原厂资料：

Specificity / Sensitivity

SUFU (C54G2) Rabbit mAb detects endogenous level of total SUFU protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Leu458 of human SUFU.

Background

SUFU (Suppressor of Fused) was identified in Drosophila as a suppressor of the Fused (Fu) kinase that is essential for Hedgehog signaling during embryonic pattern formation (1). SUFU suppresses Hedgehog signaling by regulating the localization of the transcription factors Gli and Ci (2,3). In Drosophila, SUFU may also positively regulate Hedgehog signaling depending on SUFU protein levels and Hedgehog signal intensity (4). SUFU may function as a tumor suppressor as inactivation and loss of heterozygosity of SUFU is associated with human rhabdomyosarcomas and medulloblastomas (5,6). Deletion of SUFU in mice results in embryonic lethality, while heterozygotes exhibit developmental defects characteristic of basal cell nevus syndrome. This aberrant developmental pathway is attributed to ligand-independent activation of Hedgehog signaling (7). GSK-3β binds and phosphorylates SUFU in vitro and additional information predicts that GSK-3β may positively regulate Hedgehog signaling through modification of SUFU (8).

1. Pham, A. et al. (1995) Genetics 140, 587-598.
2. Barnfield, P.C. et al. (2005) Differentiation 73, 397-405.
3. Méthot, N. and Basler, K. (2000) Development 127, 4001-4010.
4. Dussillol-Godar, F. et al. (2006) Dev. Biol. 291, 53-66.
5. Tostar, U. et al. (2006) J. Pathol. 208, 17-25.
6. Taylor, M.D. et al. (2002) Nat. Genet. 31, 306-310.
7. Svärd, J. et al. (2006) Dev. Cell. 10, 187-197.
8. Takenaka, K. et al. (2007) Biochem. Biophys. Res. Commun. 353, 501-508.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150
mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02%
sodium azide. Store at –20°C. Do not aliquot the antibody.