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Phospho-Tuberin (Thr927) Rabbit pAb (bs-3445R)  
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產(chǎn)品編號 bs-3445R
英文名稱 Phospho-Tuberin (Thr927) Rabbit pAb
中文名稱 磷酸化馬鈴薯球蛋白(結節(jié)性硬化)抗體
別    名 Tuberin(phospho T927); Tuberin(phospho Thr927); p-Tuberin(Thr927); phospho-TSC2(Thr927); P-TSC2(Thr927); TSC2; FLJ43106; LAM; TSC2_HUMAN; TSC4; Tuberous sclerosis 2; Tuberous sclerosis 2 protein; Tuberous sclerosis 2 protein homolog.  
產(chǎn)品類型 磷酸化抗體 
研究領域 腫瘤  細胞生物  免疫學  細胞表面分子  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human,Mouse,Rat (predicted: Rabbit,Pig,Cow,Chicken,Dog,Horse)
產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=2ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 200 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated Synthesised phosphopeptide derived from human Tuberin around the phosphorylation site of Thr927: DD(p-T)PE 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq].

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.

SWISS:
P49815

Gene ID:
7249

Database links:

Entrez Gene: 7249 Human

Entrez Gene: 22084 Mouse

Entrez Gene: 24855 Rat

Omim: 191092 Human

SwissProt: P49815 Human

SwissProt: Q61037 Mouse

SwissProt: P49816 Rat

Unigene: 90303 Human

Unigene: 30435 Mouse

Unigene: 5875 Rat



Tuberin 為180kD-220的蛋白產(chǎn)生于結節(jié)性硬化(TSC-2)位于染色體16的基因。結節(jié)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,在許多組織與器官中描述為錯構瘤。Tuberin被認為在GTPase活動性蛋白中起作用調節(jié)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(shù)組織中,而在皮層神經(jīng)元、小腦浦肯野氏細胞、脊索運動神經(jīng)元、胰島B細胞、心肌、腎臟與皮膚的小血管中表達增加。
產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Phospho-Tuberin (Thr927)) Polyclonal Antibody, Unconjugated (bs-3445R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Phospho-Tuberin (Thr927)) Polyclonal Antibody, Unconjugated (bs-3445R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Phospho-Tuberin (Thr927)) Polyclonal Antibody, Unconjugated (bs-3445R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Blank control(black line):Hela. Primary Antibody (green line): Rabbit Anti-Phospho-Tuberin (Thr927) antibody (bs-3445R) Dilution:2ug/Test; Secondary Antibody(white blue line): Goat anti-rabbit IgG-AF488 Dilution: 0.5ug/Test. Isotype control(orange line): Normal Rabbit IgG Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at -20℃, The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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