97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
国产精品久久热啪啪片,久久久久久爱,97福利
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Hamartin/Cy3 Conjugated antibody (bs-3837R-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-3837R-Cy3
英文名稱 Rabbit Anti-Hamartin/Cy3 Conjugated antibody
中文名稱 Cy3標記的結節性硬化癥蛋白1抗體
別    名 LAM; TSC1; Tuberous sclerosis 1 protein; TSC1_HUMAN; KIAA0243; TSC; Tuberous sclerosis 1.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  細胞周期蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, )
產品應用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 130 kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Hamartin C-terminus
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.
產品介紹 background:
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. 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.

Database links:

Entrez Gene: 7248 Human

Entrez Gene: 64930 Mouse

Entrez Gene: 60445 Rat

Omim: 605284 Human

SwissProt: Q92574 Human

SwissProt: Q9EP53 Mouse

SwissProt: Q9Z136 Rat

Unigene: 370854 Human

Unigene: 224354 Mouse

Unigene: 205837 Rat



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

????結節性硬化癥為常染色體顯性遺傳,也常見散發病例。是腫瘤抑制基因,基因產物分別為Hamartin和tuberin,兩者均調節細胞生長。
????結節性硬化癥(tuberous sclerosis)又稱結節性腦硬化,Bourneville病。本病可歸類于神經皮膚綜合征(亦稱斑痣性錯構瘤病),是源于外胚層的器官發育異常所致,病變累及神經系統、皮膚和眼,也可累及中胚層,內胚層器官如心、肺、骨,腎和胃腸等。皮脂腺瘤是皮膚神經末梢、增生的結締組織和血管組成,視網膜可見膠質瘤、神經節細胞瘤,心、腎、肺、肝臟等也可發生腫瘤。
????而神經膠質增生性硬化結節廣泛發生于大腦皮質、白質、基底節和室管膜下,常伴鈣質沉積,可出現一位癥及血管增生等,出現癲癇發作及智能減退為特征。
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 亚洲欧美闷骚少妇影院 | 亚洲精品午夜视频 | 免费黄色网址在线观看 | 狠狠色婷婷久久一区二区三区91 | 亚洲av国产国产久青草 | 91亚洲区| 精品国产片 | 国产精品对白一区二区三区 | 西西最大胆日本无码视频 | 日韩亚州 | 国产不卡视频一区 | 99精品成人无码A片观看 | 一区精品视频 | 大地资源第二页中文高清版 | 精品久久AⅤ人妻中文字幕 国产高清无码黄片亚洲大尺度视频 | 色综合久久久无码网中文 | www.97爱 | 久久美利坚合众国AV无码 | 精品成人18| 国内精品国产三级国产 | 九九视频免费观看 | 99热成人精品热久久6网站 | 裸体久久女人亚洲精品 | 久久久久人妻一区精品 | 国产在线午夜卡精品影院 | 天天躁日日躁狠狠躁2021a1 | 国产欧美色一区二区三区 | 久久艹视频 | 九九欧美 | 国内精品福利 | 欧美性受xxxx| 麻豆精品videohd4k| 国产成人精品久久二区二区 | 91av一二三区| 日本免费黄色网 | 欧美黑人添添高潮a片www | 一区二区三区四区影院 | 小泽玛利亚一区二区在线 | 欧美久久一级特黄毛片 | 欧美性猛交xxxx乱大交免费看 | 色综合久久综合网 |