FLCN (D14G9) Rabbit mAb

• 产品编号：CST-3697S      品牌：CST       原厂货号：3697S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

FLCN (D14G9) Rabbit mAb兔单抗可以检测内源性总FLCN 水平。该单克隆抗体是采用与人类FLCN氨基末端相对应的重组蛋白免疫动物而生产的。卵巢滤泡激素（FLCN）是BHD (Birt-Hogg-Dube)基因编码，其在BHD综合症里发生了改变。BHD综合症的特点是出现良性结缔组织肿瘤，也被称为纤维性毛囊瘤，肾肿瘤和肺囊肿（1）。TSC2， PTEN 和LKB1引起的错构瘤所致错乱与BHD具有相似的临床特征，表明FLCN可能在通过mTOR通路营养和能量的感知中起着重要的作用（2）。这种模式被很多研究所支持，如BHD的下调和mTOR相关S6核糖体蛋白的磷酸化减少之间具有直接的联系（3）。 TSC1 或 TSC2的突变都活导致mＴＯＲ活性的升高（4），然而，在酵母中敲除TSC2 和 BHD同源基因则在mTOR信号和氨基酸体内平衡具有相反的效应（5）。BHD敲除的小鼠会发生膀胱和肾细胞肿瘤，与伴随低水平S6核糖体蛋白磷酸化的BHD病人身上发现的相似（3）。基于以上研究，mTOR信号水平的异常升高和降低可能会引起肾细胞肿瘤形成。

1. Nickerson, M.L. et al. (2002) Cancer Cell 2, 157-64.
2. Baba, M. et al. (2006) Proc Natl Acad Sci USA 103, 15552-7.
3. Hartman, T.R. et al. (2009) Oncogene 28, 1594-604.
4. Kwiatkowski, D.J. (2003) Cancer Biol Ther 2, 471-6.
5. van Slegtenhorst, M. et al. (2007) J Biol Chem 282, 24583-90.

原厂资料：

Specificity / Sensitivity

FLCN (D14G9) Rabbit mAb detects endogenous levels of total FLCN protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with recombinant protein corresponding to the amino terminus of human FLCN.

Background

The protein folliculin (FLCN) is encoded by the BHD (Birt-Hogg-Dube) gene that is altered in BHD Syndrome, a disorder characterized by the presence of benign connective tissue tumors known as fibrofolliculomas, renal tumors and lung cysts (1). Clinical similarities between BHD and hamartoma-producing disorders caused by Tsc2, PTEN and LKB1 gene mutations indicate that FLCN might also be important in nutrient and energy sensing through the mTOR pathway (2). This model is supported by studies demonstrating a direct correlation between the down regulation of BHD and a reduction in mTOR associated phosphorylation of S6 ribosomal protein (3). Mutation of either the TSC1 or TSC2 gene results in elevated mTOR activity (4) while deletion of the Tsc2 and BHD homologs in yeast have opposing effects on both mTOR signaling and amino acid homeostasis (5). BHD knock-out mice develop cysts and renal cell tumors similar to those found in BHD patients along with low levels of phosphorylated S6 ribosomal protein (3). Based on these finding, it appears that either abnormally high or abnormally low levels of mTOR signaling might contribute to renal cell carcinogenesis.

1. Nickerson, M.L. et al. (2002) Cancer Cell 2, 157-64.
2. Baba, M. et al. (2006) Proc Natl Acad Sci USA 103, 15552-7.
3. Hartman, T.R. et al. (2009) Oncogene 28, 1594-604.
4. Kwiatkowski, D.J. (2003) Cancer Biol Ther 2, 471-6.
5. van Slegtenhorst, M. et al. (2007) J Biol Chem 282, 24583-90.

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.