中文名稱(chēng)  磷酸化性硬化癥蛋白1抗體品牌 

英文名稱(chēng)  Anti-phospho-TSC1(Ser505)

別    名 TSC1(phospho S505); LAM; TSC1; Tuberous sclerosis 1 protein.

產(chǎn)品屬性：

磷酸化性硬化癥蛋白1抗體品牌 濃    度  1mg/1ml

規(guī) 格  0.1ml/100μg

抗體來(lái)源  Rabbit

克隆類(lèi)型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit

產(chǎn)品類(lèi)型   一抗 磷酸化抗體  

研究領(lǐng)域     免疫學(xué) 染色質(zhì)和核信號(hào)

蛋白分子量  predicted molecular weight: 128kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated Synthesised phosphopeptide derived from human TSC1 around the phosphorylation site of Ser505 [FD(p-S)PF]

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

磷酸化性硬化癥蛋白1抗體品牌 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 Hamartin, or TSC1, is a suspected tumor suppressor implicated in the disease tuberous sclerosis 1. It is a negative regulator of cell division controlling the transition from G0/G1 to S phase, and it seems to act through the phosphatidylinositol 3 kinase/Akt pathway. TSC1 interacts with tuberin m(TSC2), which is thought to be a GAP (GTPase Activating Protein) for the Rap1 and Rab5 small G Proteins. The Hamartin/Tuberin complex has been shown to inhibit mTor. Hamartin has also been shown to interact with ERM (Ezrin-Radixin-Moesin) proteins and with F-actin, suggesting a role for TSC proteins in modulation of cell adhesion and morphology.

Function : In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling.

Subunit : Interacts with TSC2, leading to stabilize TSC2. In the absence of TSC2, TSC1 self-aggregates. Interacts with DOCK7. Interacts with FBXW5 and TBC1D7.

Subcellular Location : Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes.

Tissue Specificity : Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells.

Post-translational modifications : Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR.

DISEASE : Defects in TSC1 are the cause of tuberous sclerosis type 1 (TSC1) [MIM:191100]. It is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TS1C 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 TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.

性硬化癥為常染色體顯性遺傳，也常見(jiàn)散例。是抑制基因，基因產(chǎn)物分別為Hamartin和tuberin，兩者均調(diào)節(jié)細(xì)胞生長(zhǎng)。

性硬化癥(tuberous sclerosis)又稱(chēng)性腦硬化，Bourneville病。本病可歸類(lèi)于神經(jīng)皮膚綜合征(亦稱(chēng)斑痣性錯(cuò)構(gòu)瘤病)，是源于外胚層的器官發(fā)育異常所致，累及、皮膚和眼，也可累及中胚層，內(nèi)胚層器官如心、肺、骨，腎和胃腸等。皮脂腺瘤是皮膚神經(jīng)末梢、增生的結(jié)締組織和組成，視網(wǎng)膜可見(jiàn)、神經(jīng)節(jié)細(xì)胞瘤，心、腎、肺、*等也可發(fā)生。

而神經(jīng)膠質(zhì)增生性硬化廣泛發(fā)生于大腦皮質(zhì)、白質(zhì)、基底節(jié)和室管膜下，常伴鈣質(zhì)沉積，可出現(xiàn)一位癥及增生等，出現(xiàn)發(fā)作及為特征。

實(shí)驗(yàn)流程：

全、新、優(yōu)、品、好四大特點(diǎn)：

磷酸化性硬化癥蛋白1抗體品牌 全：公司提供上萬(wàn)種產(chǎn)品，涵蓋了生物試劑，elisa試劑盒，標(biāo)準(zhǔn)品，培養(yǎng)基，原裝耗材，抗體、培養(yǎng)基、ATCC細(xì)胞等，基本上各種科研所需產(chǎn)品在我司都能找到。

新：產(chǎn)品更新速度較快，基本上每周都有新產(chǎn)品出現(xiàn)。

優(yōu)：產(chǎn)品質(zhì)量好，投訴比較少。

好：我公司具有優(yōu)質(zhì)的技術(shù)團(tuán)隊(duì)，產(chǎn)品一旦售出，實(shí)驗(yàn)過(guò)程中遇到困難可提供在線技術(shù)咨詢。使您使用產(chǎn)品時(shí)沒(méi)有任何的后顧之憂。

技術(shù)外包服務(wù):

★磷酸化性硬化癥蛋白1抗體品牌 分子生物學(xué)：質(zhì)粒抽提、PCR、Q-PCR、RT-PCR、分子生物學(xué)：基因合成、引物合成、基因測(cè)序、載體構(gòu)建等

★蛋白工程：原核、哺乳動(dòng)物蛋白表達(dá)系統(tǒng)等

★病毒包裝：腺病毒、慢病毒等

★抗體工程：磁珠分選、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★細(xì)胞工程：細(xì)胞表型分析(凋亡、增殖、周期、遷移、侵襲、修復(fù)、克隆形成)、細(xì)胞培養(yǎng)、細(xì)胞膜制備、穩(wěn)定細(xì)胞株構(gòu)建、細(xì)胞RNAi技術(shù)等等。

正在熱銷(xiāo)的相關(guān)產(chǎn)品：

Leptin 瘦素(抗原)Multi-class antibodies規(guī)格： 0.5mg

Anti-CD2 CD2抗體Multi-class antibodies規(guī)格： 0.2ml

Rhesus antibody Rh PAI-2/SerpinB2 纖溶酶原激活物抑制因子2抗體(血漿酶原激活酶抑制因子-2) 規(guī)格 0.1ml

COQ7 Kit Human 人 COQ7 ELISA配對(duì)抗體 ELISA

Tsg101 英文名稱(chēng)： 易感基因101蛋白抗體 0.1ml

C6orf150 英文名稱(chēng)： 6號(hào)染色體開(kāi)放閱讀框150抗體 0.1ml

Anti-CD2 CD2抗體Multi-class antibodies規(guī)格： 0.2ml

FAS/CD95 ELISA Kit 大鼠凋亡相關(guān)因子Multi-class antibodies規(guī)格： 48T

Anti-APPL1/FITC 熒光素標(biāo)記抗銜接因子蛋白含pH域磷酸酪氨酸結(jié)合域和亮氨酸拉鏈基元1抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Rhesus antibody Rh FAM40A FAM家族蛋白40A抗體 規(guī)格 0.2ml

MECF(ouse eosinophil chemotactic factor) ELISA Kit 小鼠嗜酸粒細(xì)胞趨化因子 96T

MIA2 英文名稱(chēng)： 抑制活性蛋白2抗體 0.2ml

ALDH1B1 英文名稱(chēng)： 乙醛脫氫酶5抗體 0.1ml

Anti-APPL1/FITC 熒光素標(biāo)記抗銜接因子蛋白含pH域磷酸酪氨酸結(jié)合域和亮氨酸拉鏈基元1抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Anti- Alpha-Synuclein/FITC 熒光素標(biāo)記核突觸蛋白-α抗體IgGMulti-class antibodies規(guī)格： 0.2ml

HIV gp120 病毒(抗原)Multi-class antibodies規(guī)格： 0.5mg

一種抑制基因 Anti-PTEN/MMAC1 0.1ml

SDCCAG10 英文名稱(chēng)： 抗原10抗體 0.2ml

ERCC6L 英文名稱(chēng)： 發(fā)育相關(guān)蛋白ERCC6L抗體(乙醇致畸因子) 0.2ml

Rhesus antibody Rh Phospho-Met(Tyr1234/1235) 磷酸化肝細(xì)胞生長(zhǎng)因子受體(原癌基因)抗體 規(guī)格 0.1ml

HIV gp120 病毒(抗原)Multi-class antibodies規(guī)格： 0.5mg

大鼠繆勒管抑制物質(zhì)/抗繆勒管激素(MIS/AMH)ELISA試劑盒 ，英文名： MIS/AMH ELISA Kit

人細(xì)胞間粘附分子2(ICAM-2/CD102)ELISA檢測(cè)試劑盒Humaniercellularadhesionmolecule2,ICAM-2ELISAKit 96T/48T

鴨ELISA試劑盒

CLIAKitforPAIELISAKit大鼠纖溶酶原激活物抑制因子規(guī)格：48T/96T

水蛭素(hirudin)纖維蛋白檢測(cè)法(Fibrometer)分析試劑盒50次

ELISAKitCys-C人半胱氨酸蛋白酶抑制劑/胱抑素C規(guī)格：48T/96T

大鼠層粘連蛋白β1(LAMβ1)ELISA試劑盒 ，英文名： LAMβ1 ELISA Kit

Human plasmin inhibitor, alpha 2 (2-PI) ELISA Kit 人α2纖溶酶抑制物(α2-PI)ELISA試劑盒

Ratsolubleplateletendothelialcelladhesionmolecule1,sPECAM-1ELISAKit 大鼠可溶性血小板內(nèi)皮細(xì)胞粘附分子1(sPECAM-1/sCD31)ELISA試劑盒 96T/48T 進(jìn)口分裝

CLIAKitforC-Peptide(MouseC-Peptide)ELISAKit小鼠C肽規(guī)格：48T/96T

細(xì)胞樣品細(xì)胞色素C氧化酶活性測(cè)定試劑盒20/50/100次

RatCross-linkedCarboxy-terminaltelopeptideoftypeⅠcollagen,ⅠCTPELISAKit大鼠Ⅰ型膠原交聯(lián)羧基末端肽(ⅠCTP)ELISA試劑盒規(guī)格：96T/48T

磷酸化性硬化癥蛋白1抗體品牌 大鼠第八因子相關(guān)抗原(FⅧAg)ELISA試劑盒 ，英文名： FⅧAg ELISA Kit

Porcine hyaluronic acid (HA) ELISA Kit 豬透明質(zhì)酸(HA)ELISA試劑盒

馬鈴薯特定基因序列(PATA)核酸檢測(cè)試劑盒 48T

CLIAKitforLSP(Humanliverspecificlipoprotein)ELISAKit人抗肝特異性脂蛋白抗體規(guī)格：48T/96T

通用AP酶聯(lián)檢測(cè)封閉溶液100毫升

ELISAKitIgY雞卵黃免疫球蛋白規(guī)格：48T/96T