PTCH1 (C53A3) Rabbit mAb

• 产品编号：CST-2468S      品牌：CST       原厂货号：2468S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

PTCH1(C53A3)Rabbit mAb兔源单抗检测转染的PTCH1蛋白，此抗体也通过Western blot分析检测到被免疫沉淀的内源PTCH1。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源PTCH1蛋白邻近Pro1307 氨基酸残基序列一致。Patched1 and 2 (PTCH1 and PTCH2)是十二次跨膜蛋白，起着对Hedgehog家族蛋白成员接受的受体作用（1-4）。Hedgehog蛋白不在时，PTCH抑制活性信号受体Smoothened (Smo)，这样Hedgehog信号通路处于关闭状态（5,6）。去激活突变，修复PTCH1抑制Smo的能力，被频繁发现在痣样基底细胞癌综合征病人身上（7,8）。PTCH蛋白有固醇传感结构域（SSD）,也在一些胆固醇稳态中起作用的蛋白中发现，如HMGCR（3羟基3甲戊二酰辅酶A还原酶）和SCAP （甾醇调节成分绑定蛋白剪切激活蛋白）。但是，SSD在Patched蛋白作用还是未知（9,10）。PTCH1自身是Hedgehog信号通路的靶标蛋白（11），上调的PTCH1表达作为Hedgehog途径激活的替代标志物（12-14）。

1. Stone, D.M. et al. (1996) Nature 384, 129-134.
2. Chen, Y. and Struhl, G. (1996) Cell 87, 553-563.
3. Motoyama, J. et al. (1998) Nat. Genet. 18, 104-106.
4. Smyth, I. et al. (1999) Hum. Mol. Genet. 8, 291-297.
5. Ingham, P.W. and McMahon, A.P. (2001) Genes Dev. 15, 3059-3087.
6. McMahon, A.P. et al. (2003) Curr. Top. Dev. Biol. 53, 1-114.
7. Hahn, H. et al. (1996) Cell 85, 841-851.
8. Johnson, R.L. et al. (1996) Science 272, 1668-1671.
9. Kuwabara, P.E. and Labouesse, M. (2002) Trends Genet. 18, 193-201.
10. Chang, T.Y. et al. (2006) Annu. Rev. Cell Dev. Biol. 22, 129-157.
11. Agren, M. et al. (2004) Gene 330, 101-114.
12. Watkins, D.N. et al. (2003) Nature 422, 313-317.
13. Berman, D.M. et al. (2003) Nature 425, 846-851.
14. Karhadkar, S.S. et al. (2004) Nature 431, 707-712.

原厂资料：

Specificity / Sensitivity

PTCH1 (C53A3) Rabbit mAb detects transfected levels of PTCH1. This antibody can also detect endogenous levels of PTCH1 through immunoprecipitation and followed by Western blot analysis.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro1307 of human PTCH1.

Background

Patched1 and 2 (PTCH1 and PTCH2) are twelve-pass transmembrane proteins that function as the receiving receptors for members of the Hedgehog family of proteins (1-4). In the absence of Hedgehog proteins, PTCH suppresses the otherwise constitutively active signaling receptor Smoothened (Smo) so that the Hedgehog signaling pathway is in the off state (5,6). Deactivating mutations that impair the ability of PTCH1 to suppress Smo are frequently found in patients with nevoid basal cell carcinoma syndrome (7,8). PTCH proteins have a sterol-sensing domain (SSD) also found in several proteins that function in cholesterol homeostasis, such as HMGCR (3-hydroxy-3-methylglutaryl coenzyme A-reductase) and SCAP (sterol regulatory element-binding protein-cleavage activating protein). However, the role of the SSD in Patched proteins is not clear (9,10).PTCH1 itself is a target of Hedgehog signaling (11), with elevated PTCH1 expression as a surrogate marker for Hedgehog pathway activation (12-14).

1. Stone, D.M. et al. (1996) Nature 384, 129-134.
2. Chen, Y. and Struhl, G. (1996) Cell 87, 553-563.
3. Motoyama, J. et al. (1998) Nat. Genet. 18, 104-106.
4. Smyth, I. et al. (1999) Hum. Mol. Genet. 8, 291-297.
5. Ingham, P.W. and McMahon, A.P. (2001) Genes Dev. 15, 3059-3087.
6. McMahon, A.P. et al. (2003) Curr. Top. Dev. Biol. 53, 1-114.
7. Hahn, H. et al. (1996) Cell 85, 841-851.
8. Johnson, R.L. et al. (1996) Science 272, 1668-1671.
9. Kuwabara, P.E. and Labouesse, M. (2002) Trends Genet. 18, 193-201.
10. Chang, T.Y. et al. (2006) Annu. Rev. Cell Dev. Biol. 22, 129-157.
11. Agren, M. et al. (2004) Gene 330, 101-114.
12. Watkins, D.N. et al. (2003) Nature 422, 313-317.
13. Berman, D.M. et al. (2003) Nature 425, 846-851.
14. Karhadkar, S.S. et al. (2004) Nature 431, 707-712.

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.