TSC1 抗体 (AA 401-430)
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Quick Overview for TSC1 抗体 (AA 401-430) (ABIN3029277)
抗原
See all TSC1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 401-430
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纯化方法
- Antigen affinity purified
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免疫原
- A portion of amino acids 401-430 from the human protein was used as the immunogen for this Hamartin antibody.
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亚型
- Ig Fraction
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应用备注
- Titration of the Hamartin antibody may be required due to differences in protocols and secondary/substrate sensitivity.\. Western blot: 1:1000,IHC (Paraffin): 1:10-1:50,Immunofluorescence: 1:10-1:50
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- In 1X PBS, pH 7.4, with 0.09 % sodium azide
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Aliquot the Hamartin antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
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- TSC1 (Tuberous Sclerosis 1 (TSC1))
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别名
- Hamartin
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背景
- Implicated as a tumor suppressor. May have a function in vesicular transport. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Defects in TSC1 are the cause of tuberous sclerosis complex (TSC). The molecular basis of TSC is a functional impairement of the hamartin-tuberin complex. TSC is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TSC 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). 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.
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UniProt
- Q92574
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途径
- RTK signaling, AMPK Signaling, Regulation of Cell Size, Tube Formation
抗原
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