| 產(chǎn)品編號 | bs-3586R-BF647 |
| 英文名稱(chēng) | Rabbit Anti-Tuberin/BF647 Conjugated antibody |
| 中文名稱(chēng) | BF647標記的馬鈴薯球蛋白(結節性硬化)抗體 |
| 別 名 | TSC2; FLJ43106; LAM; OTTHUMP00000158940; OTTHUMP00000198394; OTTHUMP00000198395; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2; Tuberous sclerosis 2 protein; Tuberous sclerosis 2 protein homolog. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 腫瘤 細胞生物 免疫學(xué) 細胞表面分子 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, ) |
| 產(chǎn)品應用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 200kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Tuberin |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: Tuberin, or TSC2 (Tuberous sclerosis complex), is implicated as a tumor suppressor. It may function in vesicular transport, and may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between hamartin (TSC1) and tuberin may facilitate vesicular docking. It specifically stimulates the intrinsic GTPase activity of the Ras related protein RAP1A and RAB5, suggesting a possible mechanism for its role in regulating cellular growth. Mutations in tuberin lead to constitutive activation of RAP1A in tumors. At least three isoforms of Tuberin exist. Function: In complex with TSC1, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Acts as a GTPase-activating protein (GAP) for the small GTPase RHEB, a direct activator of the protein kinase activity of mTORC1. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. Stimulates weakly the intrinsic GTPase activity of the Ras-related proteins RAP1A and RAB5 in vitro. Mutations in TSC2 lead to constitutive activation of RAP1A in tumors. Subunit: Interacts with TSC1 and HERC1; the interaction with TSC1 stabilizes TSC2 and prevents the interaction with HERC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8. Interacts with DAPK1. Subcellular Location: Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes. Tissue Specificity: Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta. Post-translational modifications: Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1. Phosphorylation at Ser-939 and Thr-1462 by PKB/AKT1 is induced by growth factor stimulation. Phosphorylation by AMPK activates it and leads to negatively regulates the mTORC1 complex. Phosphorylated at Ser-1798 by RPS6KA1; phosphorylation inhibits TSC2 ability to suppress mTORC1 signaling. Phosphorylated by DAPK1. DISEASE: Defects in TSC2 are the cause of tuberous sclerosis type 2 (TSC2) [MIM:613254]. TSC2 is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. It is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination). Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes. Defects in TSC2 are a cause of lymphangioleiomyomatosis (LAM) [MIM:606690]. LAM is a progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex. Similarity: Contains 1 Rap-GAP domain. Database links: Entrez Gene: 7249 Human Entrez Gene: 22084 Mouse Omim: 191092 Human SwissProt: P49815 Human SwissProt: Q61037 Mouse Unigene: 90303 Human Unigene: 30435 Mouse Unigene: 5875 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Tuberin 為180kD-220的蛋白產(chǎn)生于結節性硬化（TSC-2）位于染色體16的基因。結節性硬化為常染色體疾病已知為斑痣性錯構瘤病其特征為廣泛性發(fā)展呈良性生長(cháng)，在許多組織與器官中描述為錯構瘤。Tuberin被認為在GTPase活動(dòng)性蛋白中起作用調節細胞內吞與作為腫瘤抑制子。Tuberin低水平廣泛性表達在大多數組織中，而在皮層神經(jīng)元、小腦浦肯野氏細胞、脊索運動(dòng)神經(jīng)元、胰島B細胞、心肌、腎臟與皮膚的小血管中表達增加。 |
