| 產(chǎn)品編號(hào) | bs-5611R-BF647 |
| 英文名稱(chēng) | Rabbit Anti-Phospho-Tuberin (Ser664)/BF647 Conjugated antibody |
| 中文名稱(chēng) | BF647標(biāo)記的磷酸化結(jié)節(jié)性硬化蛋白抗體 |
| 別 名 | TSC2(phospho S664);FLJ43106; LAM; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2 protein. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 產(chǎn)品類(lèi)型 | 磷酸化抗體 |
| 研究領(lǐng)域 | 腫瘤 免疫學(xué) 神經(jīng)生物學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應(yīng) | Human, (predicted: Mouse, Rat, Dog, Pig, Horse, ) |
| 產(chǎn)品應(yīng)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 Synthesised phosphopeptide derived from human Tuberin around the phosphorylation site of Ser664 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 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: E3 ubiquitin-protein ligase that mediates the polyubiquitination of a number of proteins such as CD3D, CYP3A4, CFTR and APOB for proteasomal degradation. Component of a VCP/p97-AMFR/gp78 complex that participates in the final step of endoplasmic reticulum-associated degradation (ERAD). The VCP/p97-AMFR/gp78 complex is involved in the sterol-accelerated ERAD degradation of HMGCR through binding to the HMGCR-INSIG complex at the ER membrane and initiating ubiquitination of HMGCR. The ubiquitinated HMGCR is then released from the ER by the complex into the cytosol for subsequent destruction. Also acts as a scaffold protein to assemble a complex that couples ubiquitination, retranslocation and deglycosylation. Mediates tumor invasion and metastasis. Subunit: Interacts with RNF5. Also forms an ERAD complex containing VCP/p97, NGLY1; PSMC1; SAKS1 AND RAD23B required for coupling retrotranslocation, ubiquitination and deglycosylation (By similarity). Interacts with DRL1. Interacts (through a region distinct from the RING finger) with UBE2G2/UBC7. Component of the VCP/p97-AMFR/gp78 complex that enhances VCP/p97 binding to polyubiquitinated proteins for their degradation by the endoplasmic reticulum-associated degradation (ERAD) pathway. Interacts (via the VIM) with VCP/p97. Interacts (via its membrane domain) with INSIG1; the interaction initiates the sterol-mediated ubiquitination and degradation of HMGCR by the ERAD pathway. 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 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(jié)節(jié)性硬化（TSC-2）位于染色體16的基因。結(jié)節(jié)性硬化為常染色體疾病已知為斑痣性錯(cuò)構(gòu)瘤病其特征為廣泛性發(fā)展呈良性生長(zhǎng)，在許多組織與器官中描述為錯(cuò)構(gòu)瘤。Tuberin被認(rèn)為在GTPase活動(dòng)性蛋白中起作用調(diào)節(jié)細(xì)胞內(nèi)吞與作為腫瘤抑制子。Tuberin低水平廣泛性表達(dá)在大多數(shù)組織中，而在皮層神經(jīng)元、小腦浦肯野氏細(xì)胞、脊索運(yùn)動(dòng)神經(jīng)元、胰島B細(xì)胞、心肌、腎臟與皮膚的小血管中表達(dá)增加。 |
