Hamartin/TSC1 (D43E2) Rabbit mAb

• 产品编号：CST-6935S      品牌：CST       原厂货号：6935S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Hamartin/TSC1 (D43E2) Rabbit mAb兔单抗可以检测内源性总TSC1蛋白。单克隆抗体是采用人TSC1蛋白Val640周围残基相对应的合成肽段免疫动物生产的。结节硬化综合征（TSC）是一种常染色体显性疾病，可以引起大脑、肾脏、肺脏和皮肤的错构瘤（1）。肿瘤抑制基因TSC1和TSC2分别编码错构瘤蛋白hamartin和马铃薯球蛋白tuberlin（2,3）。hamartin和tuberlin蛋白形成复合体，并且参与很多细胞活动比如小泡运输，细胞周期G1期的调控，类固醇激素的调节，Rho的激活和神经元中间丝与细胞骨架肌动蛋白的锚定（4-9）。另外，基因，生物化学和细胞生物的研究表明hamartin/uberlin蛋白复合体的功能是作为Ras相关小G蛋白Rheb的GTP酶激活蛋白，继而抑制包括mTOR在内的雷帕霉素靶蛋白（10）。细胞缺少hamartin或tuberlin会失去对S6激酶的磷酸化抑制，导致S6核糖体蛋白对5‘端mRNA转录活性的激活。Hamartin蛋白被CDK1 (cdc2)在细胞周期的G2/M 期的 Thr417, Ser584 和Thr1047 位点磷酸化（11）。

1. Sparagana, S.P. and Roach, E.S. (2000) Curr. Opin. Neurol. 13, 115-119.
2. van Slegtenhorst, M. et al. (1997) Science 277, 805-808.
3. No authors listed. (1993) Cell 75, 1305-1315.
4. Plank, T.L. et al. (1998) Cancer Res. 58, 4766-4770.
5. Xiao, G. et al. (1997) J. Biol. Chem. 272, 6097-6100.
6. Tapon, N. et al. (2001) Cell 105, 345-355.
7. Henry, K.W. et al. (1998) J. Biol. Chem. 273, 20535-20539.
8. Lamb, R.F. et al. (2000) Nat. Cell Biol. 2, 281-287.
9. Haddad, L.A. et al. (2002) J. Biol. Chem. 277, 44180-44186.
10. Manning, B.D. and Cantley, L.C. (2003) Trends Biochem Sci. 28, 573-576.
11. Astrinidis, A. et al. (2003) J. Biol. Chem. 278, 51372-51379.

原厂资料：

Specificity / Sensitivity

Hamartin/TSC1 (D43E2) Rabbit mAb recognizes endogenous levels of total TSC1 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val640 of human TSC1.

Background

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that causes symptoms including hamartomas in brain, kidney, heart, lung and skin (1). The tumor suppressor genes TSC1 and TSC2 encode hamartin and tuberin, respectively (2,3). Hamartin and tuberin form a functional complex and are involved in numerous cellular activities such as vesicular trafficking, regulation of the G1 phase of the cell cycle, steroid hormone regulation, Rho activation and anchoring neuronal intermediate filaments to the actin cytoskeleton (4-9). The combination of genetic, biochemical and cell-biological studies demonstrate that the tuberin/hamartin complex functions as a GTPase-activating protein for the Ras-related small G protein Rheb and thus inhibits targets of rapamycin including mTOR. Cells lacking hamartin or tuberin fail to inhibit phosphorylation of S6 kinase resulting in the activation of S6 ribosomal protein's translation of 5'TOP mRNA transcripts (10). Hamartin is phosphorylated by CDK1 (cdc2) at Thr417, Ser584 and Thr1047 in cells in G2/M phase of the cell cycle (11).

1. Sparagana, S.P. and Roach, E.S. (2000) Curr. Opin. Neurol. 13, 115-119.
2. van Slegtenhorst, M. et al. (1997) Science 277, 805-808.
3. No authors listed. (1993) Cell 75, 1305-1315.
4. Plank, T.L. et al. (1998) Cancer Res. 58, 4766-4770.
5. Xiao, G. et al. (1997) J. Biol. Chem. 272, 6097-6100.
6. Tapon, N. et al. (2001) Cell 105, 345-355.
7. Henry, K.W. et al. (1998) J. Biol. Chem. 273, 20535-20539.
8. Lamb, R.F. et al. (2000) Nat. Cell Biol. 2, 281-287.
9. Haddad, L.A. et al. (2002) J. Biol. Chem. 277, 44180-44186.
10. Manning, B.D. and Cantley, L.C. (2003) Trends Biochem Sci. 28, 573-576.
11. Astrinidis, A. et al. (2003) J. Biol. Chem. 278, 51372-51379.

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