Anti-Hamartin抗体(ab25882)
Key features and details
- Rabbit polyclonal to Hamartin
- Suitable for: ICC/IF, WB
- Reacts with: Mouse
- Isotype: IgG
概述
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产品名称
Anti-Hamartin抗体
参阅全部 Hamartin 一抗 -
描述
兔多克隆抗体to Hamartin -
宿主
Rabbit -
特异性
This antibody will recognise both isoforms of TSC1. -
经测试应用
适用于: ICC/IF, WBmore details -
种属反应性
与反应: Mouse -
免疫原
Synthetic peptide - a 15 amino acid peptide from the middle region of human TSC1 (Human). Peptide available as ab95277.
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阳性对照
- EL4 cell lysate can be used as positive control
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C. -
存储溶液
pH: 7.2
Preservative: 0.02% Sodium azide
Constituent: PBS -
Concentration information loading...
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纯度
Protein A purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab25882于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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ICC/IF |
Use a concentration of 2 µg/ml.
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WB |
Use a concentration of 1 µg/ml. Detects a band of approximately 130 kDa (predicted molecular weight: 130 kDa).
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说明 |
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ICC/IF
Use a concentration of 2 µg/ml. |
WB
Use a concentration of 1 µg/ml. Detects a band of approximately 130 kDa (predicted molecular weight: 130 kDa). |
靶标
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功能
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. -
组织特异性
Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells. -
疾病相关
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. -
结构域
The C-terminal putative coiled-coil domain is necessary for interaction with TSC2. -
翻译后修饰
Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR. -
细胞定位
Cytoplasm. Membrane. At steady state found in association with membranes. - Information by UniProt
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数据库链接
- Entrez Gene: 64930 Mouse
- SwissProt: Q9EP53 Mouse
- Unigene: 224354 Mouse
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别名
- Hamartin antibody
- kiaa0243 antibody
- LAM antibody
see all
图片
实验方案
数据表及文件
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SDS download
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Datasheet download
文献 (2)
ab25882 被引用在 2 文献中.
- Hadley G et al. Investigation of the novel mTOR inhibitor AZD2014 in neuronal ischemia. Neurosci Lett 706:223-230 (2019). PubMed: 31100427
- Rosengren T et al. TSC1 and TSC2 regulate cilia length and canonical Hedgehog signaling via different mechanisms. Cell Mol Life Sci 75:2663-2680 (2018). PubMed: 29396625