TSC1 抗体 (AA 401-430)
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Quick Overview for TSC1 抗体 (AA 401-430) (ABIN652207)
抗原
See all TSC1 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- AA 401-430
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纯化方法
- This antibody is purified through a protein A column, followed by peptide affinity purification.
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免疫原
- This Hamartin (TSC1) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 401-430 amino acids from the Central region of human Hamartin (TSC1).
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亚型
- Ig Fraction
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应用备注
- IF: 1:10~50. WB: 1:1000. IHC-P: 1:10~50
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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
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) 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
- 4 °C,-20 °C
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储存方法
- Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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有效期
- 6 months
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- TSC1 (Tuberous Sclerosis 1 (TSC1))
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别名
- Hamartin (TSC1)
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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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分子量
- 129767
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基因ID
- 7248
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NCBI登录号
- NP_000359, NP_001155898, NP_001155899
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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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